A clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.
A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs.
Increased numbers of platelets in the peripheral blood. (Dorland, 27th ed)
A Janus kinase subtype that is involved in signaling from GROWTH HORMONE RECEPTORS; PROLACTIN RECEPTORS; and a variety of CYTOKINE RECEPTORS such as ERYTHROPOIETIN RECEPTORS and INTERLEUKIN RECEPTORS. Dysregulation of Janus kinase 2 due to GENETIC TRANSLOCATIONS have been associated with a variety of MYELOPROLIFERATIVE DISORDERS.
Conditions which cause proliferation of hemopoietically active tissue or of tissue which has embryonic hemopoietic potential. They all involve dysregulation of multipotent MYELOID PROGENITOR CELLS, most often caused by a mutation in the JAK2 PROTEIN TYROSINE KINASE.
A de novo myeloproliferation arising from an abnormal stem cell. It is characterized by the replacement of bone marrow by fibrous tissue, a process that is mediated by CYTOKINES arising from the abnormal clone.
Cell surface receptors that are specific for THROMBOPOIETIN. They signal through interaction with JANUS KINASES such as JANUS KINASE 2.
The number of PLATELETS per unit volume in a sample of venous BLOOD.
A humoral factor that stimulates the production of thrombocytes (BLOOD PLATELETS). Thrombopoietin stimulates the proliferation of bone marrow MEGAKARYOCYTES and their release of blood platelets. The process is called THROMBOPOIESIS.
Formation and development of a thrombus or blood clot in the blood vessel.
An alkylating agent structurally similar to MITOMYCIN and found to be effective in the treatment of leukemia and various other neoplasms in mice. It causes leukemia and thrombocytopenia in almost all human patients.
Very large BONE MARROW CELLS which release mature BLOOD PLATELETS.
An antineoplastic agent that inhibits DNA synthesis through the inhibition of ribonucleoside diphosphate reductase.
An antineoplastic agent that acts by alkylation.
A transient increase in the number of leukocytes in a body fluid.
Leukocytes with abundant granules in the cytoplasm. They are divided into three groups according to the staining properties of the granules: neutrophilic, eosinophilic, and basophilic. Mature granulocytes are the NEUTROPHILS; EOSINOPHILS; and BASOPHILS.
A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)
The process of generating thrombocytes (BLOOD PLATELETS) from the pluripotent HEMATOPOIETIC STEM CELLS in the BONE MARROW via the MEGAKARYOCYTES. The humoral factor with thrombopoiesis-stimulating activity is designated THROMBOPOIETIN.
Pathologic inclusions occurring in erythrocytes.
Enlargement of the spleen.
An increase in the total red cell mass of the blood. (Dorland, 27th ed)
Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation.
A severe sometimes chronic anemia, usually macrocytic in type, that does not respond to ordinary antianemic therapy.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
Agents that cause clotting.
A disorder of HEMOSTASIS in which there is a tendency for the occurrence of THROMBOSIS.
A myelodysplastic/myeloproliferative disorder characterized by myelodysplasia associated with bone marrow and peripheral blood patterns similar to CHRONIC MYELOID LEUKEMIA, but cytogenetically lacking a PHILADELPHIA CHROMOSOME or bcr/abl fusion gene (GENES, ABL).
The naturally occurring or experimentally induced replacement of one or more AMINO ACIDS in a protein with another. If a functionally equivalent amino acid is substituted, the protein may retain wild-type activity. Substitution may also diminish, enhance, or eliminate protein function. Experimentally induced substitution is often used to study enzyme activities and binding site properties.
The co-occurrence of pregnancy and a blood disease (HEMATOLOGIC DISEASES) which involves BLOOD CELLS or COAGULATION FACTORS. The hematologic disease may precede or follow FERTILIZATION and it may or may not have a deleterious effect on the pregnant woman or FETUS.
The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells.
Drugs or agents which antagonize or impair any mechanism leading to blood platelet aggregation, whether during the phases of activation and shape change or following the dense-granule release reaction and stimulation of the prostaglandin-thromboxane system.
Bleeding or escape of blood from a vessel.
A specialized agency of the United Nations designed as a coordinating authority on international health work; its aim is to promote the attainment of the highest possible level of health by all peoples.
A subclass of lipid-linked proteins that contain a GLYCOSYLPHOSPHATIDYLINOSITOL LINKAGE which holds them to the CELL MEMBRANE.
A subnormal level of BLOOD PLATELETS.
A vascular anomaly that is a collection of tortuous BLOOD VESSELS and connective tissue. This tumor-like mass with the large vascular space is filled with blood and usually appears as a strawberry-like lesion in the subcutaneous areas of the face, extremities, or other regions of the body including the central nervous system.
Quinazolines are heterocyclic aromatic organic compounds consisting of a benzene ring fused to a pyrazine ring, which are synthesized and used as intermediates in pharmaceuticals, particularly in the production of various drugs such as antimalarials, antihypertensives, and antitumor agents.
Cell surface proteins that bind cytokines and trigger intracellular changes influencing the behavior of cells.
Antigens that exist in alternative (allelic) forms in a single species. When an isoantigen is encountered by species members who lack it, an immune response is induced. Typical isoantigens are the BLOOD GROUP ANTIGENS.

Prognostic significance of bone marrow biopsy in essential thrombocythemia. (1/263)

BACKGROUND AND OBJECTIVE: The diagnostic and prognostic value of bone marrow biopsy (BMB) has been widely investigated in patients with chronic myeloproliferative disorders (CMPD). The present study is based on a review of the results of routine BMBs taken from 93 essential thrombocythemia (ET) patients at the time of diagnosis. DESIGN AND METHODS: The common BMB histologic parameters and clinico-hematologic variables were considered for diagnostic and prognostic purposes. Clinico-pathologic correlations were looked for univariately. Moreover, the diagnostic significance of the histologic findings was tested by means of cluster analysis. Overall survival and event-free survival were considered as prognostic endpoints. RESULTS: There were no correlations between the clinic and pathologic findings, and none of the histologic and clinical parameters was predictive of survival or the occurrence of major clinical events. Cluster analysis of the BMB findings revealed two distinct morphologic patterns: one was clearly myeloproliferative; the other had somewhat dysplastic features. The event-free and overall survival rates in the latter group were significantly worse (p = 0.0377 and p = 0.0162 respectively), with major ischemic events accounting for most of the difference in event-free survival. INTERPRETATION AND CONCLUSIONS: These results have no clearcut counterpart in the literature, but we feel that dysplastic BMB findings could be included in the definition of ET prognostic scores in order to allow therapeutic strategies to be adapted to the level of risk.  (+info)

Mother cell of megakaryocyte. (2/263)

It was attempted to describe the morphology of the most immature cell of megakaryocytic series. The megakaryocytes were observed with the electron microscope in five cases, being traced back to their immature forms. In two cases the most immature cells of megakaryocytic series were considered to be the cells which were probably identified as lymphocytes under the light microscope, but they were not lymphocytes with the electron microscope. In other two cases it was presumed that neutrophilic and megakaryocytic series were derived from morphologically similar immature cells, since the most immature cells of neutrophilic and megakaryocytic series were not distinguished when they were traced back to their immature forms. These findings suggest that mother cells of megakaryocytes in the adult bone marrow may be identified as lymphoid cells with the light microscope.  (+info)

Mechanism of platelet liberation. (3/263)

Megakaryocytes from 5 patients and 1 normal person were observed electronmicroscopically. In some pathologic states platelets seemed to be liberated without demarcation membrane system (DMS) and in a normal individual they seemed to be liberated independently of DMS. These findings suggest that DMS is not concerned with platelet liberation and that platelets are liberated through pseudopodia and bleb formation. In mature megakaryocytes vigorous amoeboid movement seems to exist and both pseudopodia and blebs may represent this movement. Structural similarity between surface connected system (SCS) of platelet and DMS of megakaryocyte suggests that the structure called DMS is transported as SCS into platelet.  (+info)

Artefactual serum hyperkalaemia and hypercalcaemia in essential thrombocythaemia. (4/263)

AIM: To investigate possible abnormalities of serum potassium and calcium levels in patients with essential thrombocythaemia and significant thrombocytosis. METHODS: 24 cases of essential thrombocythaemia with significant thrombocytosis (platelet count > 700 x 10(9)/litre) had serum potassium and calcium estimations performed at the time of maximum thrombocytosis before treatment, and at the time of low platelet count after treatment with cytoreductive drugs. Selected patients were further investigated with plasma sampling and estimation of ionised calcium and parathyroid hormone. RESULTS: At the time of maximum thrombocytosis six patients had serum hyperkalaemia (> 5.5 mmol/litre) and five had serum hypercalcaemia (> 2.6 mmol/litre). Following treatment and reduction of the platelet count, hyperkalaemia resolved in all cases and hypercalcaemia in four of the five cases. Mean serum potassium and calcium concentrations were raised (p < 0.0001) at maximum thrombocytosis compared with the values when the platelet count was low. Serum potassium and calcium values were significantly correlated at all stages. Measurements on plasma consistently corrected the hyperkalaemia but not the hypercalcaemia. Serum hypercalcaemia was associated with raised ionised calcium and normal parathyroid hormone concentrations. CONCLUSIONS: Essential thrombocythaemia with significant thrombocytosis is associated with serum hyperkalaemia and hypercalcaemia. The probable mechanism of hypercalcaemia is the secretion of calcium in vitro from an excessive number of abnormally activated platelets. It is thus likely that the hypercalcaemia is an artefact, as is the hyperkalaemia.  (+info)

Diagnosis of essential thrombocythemia at platelet counts between 400 and 600x10(9)/L. Gruppo Italiano Malattie Mieloproliferative Croniche(GIMMC). (5/263)

BACKGROUND AND OBJECTIVE: Diagnostic criteria for essential thrombocythemia (ET) remain essentially negative, that is, exclusion of other myeloproliferative diseases and causes of reactive thrombocytosis. A platelet count above 600x10(9)/L is still generally considered an absolute diagnostic criterion although new protocols for positive diagnostic criteria have recently been proposed, reducing the stringency of a definite platelet limit. This study demonstrates that a platelet count 600x10(9)/L is not a reliable diagnostic criterion for ET, especially in the early stages. DESIGN AND METHODS: An ongoing retrospective study by the GIMMC analyzed 2,316 ET patients diagnosed between 1986 and 1995. Of these 2,316 patients, diagnosed according to the PVSG criteria, 68 had a platelet count 600x10(9)/L and were analyzed separately; 37 out of 68 were excluded from this analysis because of a follow-up shorter than 2 years and/or because of treatment with myelosuppressive agents. The remaining 31 patients were the subjects of our study. RESULTS: After a median follow-up of 4.56 years (range 2-9.6 years) none of the 31 patients had a spontaneous decrease of platelets to the normal range. Transformation to a different chronic myeloproliferative disorders was never observed and no patient developed a condition known to produce reactive thrombocytosis. During follow-up, 23 patients (74%) were treated with anti-aggregating drugs, mainly aspirin. The disease did not evolve into acute leukemia in any patient, 1 had a thrombotic event and none presented hemorrhagic episodes. Median platelet count during follow-up was 534x10(9)/L (range 398-997x10(9)/L). INTERPRETATION AND CONCLUSIONS: Long term follow-up has documented that our 31 patients were correctly diagnosed as having ET, although platelet count was 600x10(9)/L. Our patients were probably in a early phase of their disease and following updated PVSG criteria would have been misdiagnosed leading to incomplete recognition of the natural history of the disease. Further, because an early diagnosis could also have a clinical relevance, our results outline the need for new criteria for the diagnosis of ET. The exclusion of patients with a platelet count between 400 and 600x10(9)/L may prevent patients, nevertheless at risk of vascular complications, from being treated.  (+info)

Essential thrombocythemia transformed to acute myelogenous leukemia with t(3;17)(p24; q12), del(5)(q13q34) after treatment with carboquone and hydroxyurea. (6/263)

In 1991, a 52-year-old woman was diagnosed as having essential thrombocythemia (ET). She was doing well with continuous medication with carboquone (CQ) and subsequently hydroxyurea (HU). However, substantial leukocytosis with leukemic blast cells, anemia and thrombocytopenia developed in 1996. Analysis of peripheral blood showed 4.4 x 10(3)/microl white blood cells with 82% of leukemic blast cells. These blasts showed negative staining with myeloperoxidase by immunostaining, but the myeloperoxidase was positive by electron microscopic analysis. Cytogenetic analysis of bone marrow cells revealed a t(3;17)(p24; q12), del(5)(q13q34). On the basis of these findings, the leukemic blast cells were classified as acute myelogenous leukemia (AML:M0) in the FAB classification. The causative agent, CQ and HU in secondary leukemia from ET and chromosomal abnormality related to ET blastic crisis (BC) are discussed.  (+info)

Relevance of bone marrow features in the differential diagnosis between essential thrombocythemia and early stage idiopathic myelofibrosis. (7/263)

BACKGROUND AND OBJECTIVES: Diagnosis of essential thrombocythemia (ET) remains a challenging problem and has been predominantly established by exclusion of other thrombocythemic disorders. In this context the updated diagnostic criteria of the Polycythemia Vera Study Group (PVSG) are generally accepted, although histopathologic features of the bone marrow were only marginally considered. DESIGN AND METHODS: A retrospective evaluation was performed of 168 patients presenting with ET in accordance with the criteria of the PVSG. Analysis was focused on the discriminating impact of bone marrow morphology. RESULTS: Histopathology revealed that our cohort of patients could be divided into three distinct groups (true ET, questionable ET and false ET). These groups were characterized by certain diagnostic constellations of clinical data on admission. True ET was found in 53 patients presenting with no or a borderline splenomegaly and no relevant anemia or leuko-erythroblastic blood picture. The other patients showed clinical signs and symptoms which were more compatible with initial-prefibrotic (52 patients) or early (68 patients) idiopathic-primary myelofibrosis (IMF) with severe thrombocythemia. In true ET no significant hypercellularity of the bone marrow including myeloid precursors or an increase in reticulin fibers was detectable. Most prominent were changes of megakaryopoiesis which revealed large to giant-sized cells lacking a definite maturation defect. Their appearance in true ET contrasted with the clusters of abnormally differentiated, often bizarre elements of this lineage in patients with initial and early IMF (questionable or false ET). Calculation of survival disclosed a relevant disparity with a non-significant loss in life expectancy of 10.9% in true ET compared to 29.6% in questionable and 51.3% in false ET. Follow-up studies and repeated bone marrow biopsies revealed no transition into myelofibrosis in true ET, whereas this did occur in 22 of 27 patients with questionable and false ET. In the latter cohort bone marrow changes were accompanied by increasing anemia, splenomegaly, tear-drop poikilocytosis and reduction of the platelet count consistent with IMF. INTERPRETATION AND CONCLUSIONS: A detailed evaluation of bone marrow features, in particular megakaryopoiesis is recommended to establish positive criteria for the diagnosis of ET and thus to accomplish a significant improvement of the PVSG postulates. In this context ongoing clinical trials on ET must regard pretreatment bone marrow biopsies as a major clue to diagnosis.  (+info)

Isolated spleen agenesis: a rare cause of thrombocytosis mimicking essential thrombocythemia. (8/263)

Thrombocytosis is a common feature of myeloproliferative disorders but may also result from various conditions including chronic iron deficiency, hemorrhage, chronic inflammation and splenectomy. We report two cases of secondary thrombocytosis caused by isolated and congenital asplenia, mimicking essential thrombocythemia. These two adult cases of spleen agenesis were unexpected. We conclude that in thrombocytosis without clinical evidence of splenomegaly, attentive screening of blood in search of Howell-Jolly bodies and abdominal ultrasonography should always be performed not only to detect mild spleen enlargement but also to make sure of the presence of this organ.  (+info)

Essential thrombocythemia (ET) is a myeloproliferative neoplasm (MPN), a type of blood cancer characterized by the overproduction of platelets (thrombocytosis) in the bone marrow. In ET, there is an excessive proliferation of megakaryocytes, the precursor cells that produce platelets. This leads to increased platelet counts in the peripheral blood, which can increase the risk of blood clots (thrombosis) and bleeding episodes (hemorrhage).

The term "essential" is used to indicate that the cause of this condition is not known or idiopathic. ET is primarily a disease of older adults, but it can also occur in younger individuals. The diagnosis of essential thrombocythemia requires careful evaluation and exclusion of secondary causes of thrombocytosis, such as reactive conditions, inflammation, or other myeloproliferative neoplasms.

The clinical presentation of ET can vary widely among patients. Some individuals may be asymptomatic and discovered only during routine blood tests, while others may experience symptoms related to thrombosis or bleeding. Common symptoms include headaches, visual disturbances, dizziness, weakness, numbness, or tingling in the extremities, if there are complications due to blood clots in the brain or other parts of the body. Excessive bruising, nosebleeds, or blood in the stool can indicate bleeding complications.

Treatment for essential thrombocythemia is aimed at reducing the risk of thrombosis and managing symptoms. Hydroxyurea is a commonly used medication to lower platelet counts, while aspirin may be prescribed to decrease the risk of blood clots. In some cases, interferon-alpha or ruxolitinib might be considered as treatment options. Regular follow-up with a hematologist and monitoring of blood counts are essential for managing this condition and detecting potential complications early.

Polycythemia Vera is a type of myeloproliferative neoplasm, a group of rare blood cancers. In Polycythemia Vera, the body produces too many red blood cells, leading to an increased risk of blood clots and thickening of the blood, which can cause various symptoms such as fatigue, headache, dizziness, and itching. It can also lead to enlargement of the spleen. The exact cause of Polycythemia Vera is not known, but it is associated with genetic mutations in the JAK2 gene in most cases. It is a progressive disease that can lead to complications such as bleeding, thrombosis, and transformation into acute leukemia if left untreated.

Thrombocytosis is a medical condition characterized by an abnormally high platelet count (also known as thrombocytes) in the blood. Platelets are small cell fragments that play a crucial role in blood clotting. A normal platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. Thrombocytosis is typically defined as a platelet count exceeding 450,000-500,000 platelets/µL.

Thrombocytosis can be classified into two types: reactive (or secondary) thrombocytosis and primary (or essential) thrombocytosis. Reactive thrombocytosis is more common and occurs as a response to an underlying condition, such as infection, inflammation, surgery, or certain types of cancer. Primary thrombocytosis, on the other hand, is caused by intrinsic abnormalities in the bone marrow cells responsible for platelet production (megakaryocytes), and it is often associated with myeloproliferative neoplasms like essential thrombocythemia.

While mild thrombocytosis may not cause any symptoms, higher platelet counts can increase the risk of blood clots (thrombosis) and bleeding disorders due to excessive platelet aggregation. Symptoms of thrombocytosis may include headaches, dizziness, visual disturbances, or chest pain if a blood clot forms in the brain or heart. Bleeding symptoms can manifest as easy bruising, nosebleeds, or gastrointestinal bleeding.

Treatment for thrombocytosis depends on the underlying cause and the severity of the condition. In cases of reactive thrombocytosis, treating the underlying disorder often resolves the high platelet count. For primary thrombocytosis, medications like aspirin or cytoreductive therapy (such as hydroxyurea) may be used to reduce the risk of blood clots and control platelet production. Regular monitoring of platelet counts is essential for managing this condition and preventing potential complications.

Janus Kinase 2 (JAK2) is a tyrosine kinase enzyme that plays a crucial role in intracellular signal transduction. It is named after the Roman god Janus, who is depicted with two faces, as JAK2 has two similar phosphate-transferring domains. JAK2 is involved in various cytokine receptor-mediated signaling pathways and contributes to hematopoiesis, immune function, and cell growth.

Mutations in the JAK2 gene have been associated with several myeloproliferative neoplasms (MPNs), including polycythemia vera, essential thrombocythemia, and primary myelofibrosis. The most common mutation is JAK2 V617F, which results in a constitutively active enzyme that promotes uncontrolled cell proliferation and survival, contributing to the development of these MPNs.

Myeloproliferative disorders (MPDs) are a group of rare, chronic blood cancers that originate from the abnormal proliferation or growth of one or more types of blood-forming cells in the bone marrow. These disorders result in an overproduction of mature but dysfunctional blood cells, which can lead to serious complications such as blood clots, bleeding, and organ damage.

There are several subtypes of MPDs, including:

1. Chronic Myeloid Leukemia (CML): A disorder characterized by the overproduction of mature granulocytes (a type of white blood cell) in the bone marrow, leading to an increased number of these cells in the blood. CML is caused by a genetic mutation that results in the formation of the BCR-ABL fusion protein, which drives uncontrolled cell growth and division.
2. Polycythemia Vera (PV): A disorder characterized by the overproduction of all three types of blood cells - red blood cells, white blood cells, and platelets - in the bone marrow. This can lead to an increased risk of blood clots, bleeding, and enlargement of the spleen.
3. Essential Thrombocythemia (ET): A disorder characterized by the overproduction of platelets in the bone marrow, leading to an increased risk of blood clots and bleeding.
4. Primary Myelofibrosis (PMF): A disorder characterized by the replacement of normal bone marrow tissue with scar tissue, leading to impaired blood cell production and anemia, enlargement of the spleen, and increased risk of infections and bleeding.
5. Chronic Neutrophilic Leukemia (CNL): A rare disorder characterized by the overproduction of neutrophils (a type of white blood cell) in the bone marrow, leading to an increased number of these cells in the blood. CNL can lead to an increased risk of infections and organ damage.

MPDs are typically treated with a combination of therapies, including chemotherapy, targeted therapy, immunotherapy, and stem cell transplantation. The choice of treatment depends on several factors, including the subtype of MPD, the patient's age and overall health, and the presence of any comorbidities.

Primary myelofibrosis (PMF) is a rare, chronic bone marrow disorder characterized by the replacement of normal bone marrow tissue with fibrous scar tissue, leading to impaired production of blood cells. This results in cytopenias (anemia, leukopenia, thrombocytopenia), which can cause fatigue, infection susceptibility, and bleeding tendencies. Additionally, PMF is often accompanied by the proliferation of abnormal megakaryocytes (large, atypical bone marrow cells that produce platelets) and extramedullary hematopoiesis (blood cell formation outside the bone marrow, typically in the spleen and liver).

PMF is a type of myeloproliferative neoplasm (MPN), which is a group of clonal stem cell disorders characterized by excessive proliferation of one or more types of blood cells. PMF can present with various symptoms such as fatigue, weight loss, night sweats, abdominal discomfort due to splenomegaly (enlarged spleen), and bone pain. In some cases, PMF may progress to acute myeloid leukemia (AML).

The exact cause of PMF remains unclear; however, genetic mutations are known to play a significant role in its development. The Janus kinase 2 (JAK2), calreticulin (CALR), and MPL genes have been identified as commonly mutated in PMF patients. These genetic alterations contribute to the dysregulated production of blood cells and the activation of signaling pathways that promote fibrosis.

Diagnosis of PMF typically involves a combination of clinical evaluation, complete blood count (CBC), bone marrow aspiration and biopsy, cytogenetic analysis, and molecular testing to identify genetic mutations. Treatment options depend on the individual patient's symptoms, risk stratification, and disease progression. They may include observation, supportive care, medications to manage symptoms and control the disease (such as JAK inhibitors), and stem cell transplantation for eligible patients.

Thrombopoietin receptors are a type of cell surface receptor found on megakaryocytes and platelets. They are also known as MPL (myeloproliferative leukemia virus) receptors. Thrombopoietin is a hormone that regulates the production of platelets in the body, and it binds to these receptors to stimulate the proliferation and differentiation of megakaryocytes, which are large bone marrow cells that produce platelets.

The thrombopoietin receptor is a type I transmembrane protein with an extracellular domain that contains the thrombopoietin-binding site, a single transmembrane domain, and an intracellular domain that contains several tyrosine residues that become phosphorylated upon thrombopoietin binding. This triggers a signaling cascade that leads to the activation of various downstream pathways involved in cell proliferation, differentiation, and survival.

Mutations in the thrombopoietin receptor gene have been associated with certain myeloproliferative neoplasms, such as essential thrombocythemia and primary myelofibrosis, which are characterized by excessive platelet production and bone marrow fibrosis.

A platelet count is a laboratory test that measures the number of platelets, also known as thrombocytes, in a sample of blood. Platelets are small, colorless cell fragments that circulate in the blood and play a crucial role in blood clotting. They help to stop bleeding by sticking together to form a plug at the site of an injured blood vessel.

A normal platelet count ranges from 150,000 to 450,000 platelets per microliter (µL) of blood. A lower than normal platelet count is called thrombocytopenia, while a higher than normal platelet count is known as thrombocytosis.

Abnormal platelet counts can be a sign of various medical conditions, including bleeding disorders, infections, certain medications, and some types of cancer. It is important to consult with a healthcare provider if you have any concerns about your platelet count or if you experience symptoms such as easy bruising, prolonged bleeding, or excessive menstrual flow.

Thrombopoietin (TPO) is a glycoprotein hormone that plays a crucial role in the regulation of platelet production, also known as thrombopoiesis. It is primarily produced by the liver and to some extent by megakaryocytes, which are the cells responsible for producing platelets.

TPO binds to its receptor, c-Mpl, on the surface of megakaryocytes and their precursor cells, stimulating their proliferation, differentiation, and maturation into platelets. By regulating the number of platelets in circulation, TPO helps maintain hemostasis, the process that prevents excessive bleeding after injury.

In addition to its role in thrombopoiesis, TPO has been shown to have potential effects on other cell types, including hematopoietic stem cells and certain immune cells. However, its primary function remains the regulation of platelet production.

Thrombosis is the formation of a blood clot (thrombus) inside a blood vessel, obstructing the flow of blood through the circulatory system. When a clot forms in an artery, it can cut off the supply of oxygen and nutrients to the tissues served by that artery, leading to damage or tissue death. If a thrombus forms in the heart, it can cause a heart attack. If a thrombus breaks off and travels through the bloodstream, it can lodge in a smaller vessel, causing blockage and potentially leading to damage in the organ that the vessel supplies. This is known as an embolism.

Thrombosis can occur due to various factors such as injury to the blood vessel wall, abnormalities in blood flow, or changes in the composition of the blood. Certain medical conditions, medications, and lifestyle factors can increase the risk of thrombosis. Treatment typically involves anticoagulant or thrombolytic therapy to dissolve or prevent further growth of the clot, as well as addressing any underlying causes.

Carbamazepine 10,11-epoxide, also known as carbazilquinone, is a metabolite of the anticonvulsant drug carbamazepine. It is an active metabolite that contributes to the therapeutic effect of carbamazepine in the treatment of seizures. However, it can also contribute to some of the side effects associated with carbamazepine therapy, such as liver toxicity and bone marrow suppression.

Carbamazepine is metabolized in the liver by cytochrome P450 enzymes to form carbamazepine 10,11-epoxide. This metabolite can then be further metabolized to other compounds or conjugated with glucuronic acid and excreted in the urine.

It is important to monitor patients taking carbamazepine for signs of toxicity, including liver function tests and complete blood counts, to ensure that the drug is being properly metabolized and eliminated from the body.

Megakaryocytes are large, specialized bone marrow cells that are responsible for the production and release of platelets (also known as thrombocytes) into the bloodstream. Platelets play an essential role in blood clotting and hemostasis, helping to prevent excessive bleeding during injuries or trauma.

Megakaryocytes have a unique structure with multilobed nuclei and abundant cytoplasm rich in organelles called alpha-granules and dense granules, which store various proteins, growth factors, and enzymes necessary for platelet function. As megakaryocytes mature, they extend long cytoplasmic processes called proplatelets into the bone marrow sinuses, where these extensions fragment into individual platelets that are released into circulation.

Abnormalities in megakaryocyte number, size, or function can lead to various hematological disorders, such as thrombocytopenia (low platelet count), thrombocytosis (high platelet count), and certain types of leukemia.

Hydroxyurea is an antimetabolite drug that is primarily used in the treatment of myeloproliferative disorders such as chronic myelogenous leukemia (CML), essential thrombocythemia, and polycythemia vera. It works by interfering with the synthesis of DNA, which inhibits the growth of cancer cells.

In addition to its use in cancer therapy, hydroxyurea is also used off-label for the management of sickle cell disease. In this context, it helps to reduce the frequency and severity of painful vaso-occlusive crises by increasing the production of fetal hemoglobin (HbF), which decreases the formation of sickled red blood cells.

The medical definition of hydroxyurea is:

A hydantoin derivative and antimetabolite that inhibits ribonucleoside diphosphate reductase, thereby interfering with DNA synthesis. It has been used as an antineoplastic agent, particularly in the treatment of myeloproliferative disorders, and more recently for the management of sickle cell disease to reduce the frequency and severity of painful vaso-occlusive crises by increasing fetal hemoglobin production.

Pipobroman is an antineoplastic agent, which means it is used to treat cancer. It's a type of alkylating agent, specifically a nitrogen mustard. Alkylating agents work by disrupting the DNA of cancer cells, which can prevent them from dividing and growing. Pipobroman has been used in the treatment of chronic myelogenous leukemia (CML), although it's not widely used today due to the availability of more effective treatments.

Please note that medical definitions can vary based on the source, and this definition is intended to be a general overview. Always refer to the most current prescribing information for any medication.

Leukocytosis is a condition characterized by an increased number of leukocytes (white blood cells) in the peripheral blood. A normal white blood cell count ranges from 4,500 to 11,000 cells per microliter of blood in adults. Leukocytosis is typically considered present when the white blood cell count exceeds 11,000 cells/µL. However, the definition might vary slightly depending on the laboratory and clinical context.

Leukocytosis can be a response to various underlying conditions, including bacterial or viral infections, inflammation, tissue damage, leukemia, and other hematological disorders. It is essential to investigate the cause of leukocytosis through further diagnostic tests, such as blood smears, differential counts, and additional laboratory and imaging studies, to guide appropriate treatment.

Granulocytes are a type of white blood cell that plays a crucial role in the body's immune system. They are called granulocytes because they contain small granules in their cytoplasm, which are filled with various enzymes and proteins that help them fight off infections and destroy foreign substances.

There are three types of granulocytes: neutrophils, eosinophils, and basophils. Neutrophils are the most abundant type and are primarily responsible for fighting bacterial infections. Eosinophils play a role in defending against parasitic infections and regulating immune responses. Basophils are involved in inflammatory reactions and allergic responses.

Granulocytes are produced in the bone marrow and released into the bloodstream, where they circulate and patrol for any signs of infection or foreign substances. When they encounter a threat, they quickly move to the site of infection or injury and release their granules to destroy the invading organisms or substances.

Abnormal levels of granulocytes in the blood can indicate an underlying medical condition, such as an infection, inflammation, or a bone marrow disorder.

A missense mutation is a type of point mutation in which a single nucleotide change results in the substitution of a different amino acid in the protein that is encoded by the affected gene. This occurs when the altered codon (a sequence of three nucleotides that corresponds to a specific amino acid) specifies a different amino acid than the original one. The function and/or stability of the resulting protein may be affected, depending on the type and location of the missense mutation. Missense mutations can have various effects, ranging from benign to severe, depending on the importance of the changed amino acid for the protein's structure or function.

Thrombopoiesis is the process of formation and development of thrombocytes or platelets, which are small, colorless cell fragments in our blood that play an essential role in clotting. Thrombopoiesis occurs inside the bone marrow, where stem cells differentiate into megakaryoblasts, then progressively develop into promegakaryocytes and megakaryocytes. These megakaryocytes subsequently undergo a process called cytoplasmic fragmentation to produce platelets.

The regulation of thrombopoiesis is primarily controlled by the hormone thrombopoietin (TPO), which is produced mainly in the liver and binds to the thrombopoietin receptor (c-Mpl) on megakaryocytes and their precursors. This binding stimulates the proliferation, differentiation, and maturation of megakaryocytes, leading to an increase in platelet production.

Abnormalities in thrombopoiesis can result in conditions such as thrombocytopenia (low platelet count) or thrombocytosis (high platelet count), which may be associated with bleeding disorders or increased risk of thrombosis, respectively.

Erythrocyte inclusions refer to the presence of abnormal structures or substances within red blood cells (erythrocytes). These inclusions can be composed of various materials such as proteins, pigments, or foreign bodies. They may be seen in a variety of medical conditions and can provide important diagnostic clues.

Some examples of erythrocyte inclusions include:

1. Howell-Jolly bodies: small remnants of nuclear material left behind after the red blood cell matures. They are typically seen in individuals with an absent or nonfunctional spleen.
2. Heinz bodies: denatured hemoglobin that forms clumps within the red blood cells. They can be seen in conditions such as hemolytic anemia, G6PD deficiency, and exposure to certain drugs or toxins.
3. Pappenheimer bodies: aggregates of iron-containing proteins called ferritin or hemosiderin. They are typically seen in conditions associated with increased red blood cell destruction, such as thalassemia or lead poisoning.
4. Basophilic stippling: small, basophilic (blue-staining) granules within the red blood cells. They can be seen in various conditions, including lead poisoning, megaloblastic anemias, and certain inherited disorders.
5. Parasites: organisms such as malaria or babesia that infect and multiply within the red blood cells.

The detection of erythrocyte inclusions typically requires specialized testing, such as peripheral blood smears stained with specific dyes to highlight the abnormal structures. The presence and type of inclusions can help diagnose certain medical conditions and guide appropriate treatment.

Splenomegaly is a medical term that refers to an enlargement or expansion of the spleen beyond its normal size. The spleen is a vital organ located in the upper left quadrant of the abdomen, behind the stomach and below the diaphragm. It plays a crucial role in filtering the blood, fighting infections, and storing red and white blood cells and platelets.

Splenomegaly can occur due to various underlying medical conditions, including infections, liver diseases, blood disorders, cancer, and inflammatory diseases. The enlarged spleen may put pressure on surrounding organs, causing discomfort or pain in the abdomen, and it may also lead to a decrease in red and white blood cells and platelets, increasing the risk of anemia, infections, and bleeding.

The diagnosis of splenomegaly typically involves a physical examination, medical history, and imaging tests such as ultrasound, CT scan, or MRI. Treatment depends on the underlying cause and may include medications, surgery, or other interventions to manage the underlying condition.

Polycythemia is a medical condition characterized by an abnormal increase in the total red blood cell (RBC) mass or hematocrit (the percentage of RBCs in the blood). This results in a higher-than-normal viscosity of the blood, which can lead to various complications such as impaired circulation, increased risk of blood clots, and reduced oxygen supply to the tissues.

There are two main types of polycythemia: primary and secondary. Primary polycythemia, also known as polycythemia vera, is a rare myeloproliferative neoplasm caused by genetic mutations that lead to excessive production of RBCs in the bone marrow. Secondary polycythemia, on the other hand, is a reactive condition triggered by various factors such as chronic hypoxia (low oxygen levels), high altitude, smoking, or certain medical conditions like sleep apnea, heart disease, or kidney tumors.

Symptoms of polycythemia may include fatigue, headaches, dizziness, shortness of breath, itching, and a bluish or reddish tint to the skin (cyanosis). Treatment depends on the underlying cause and severity of the condition and may involve phlebotomy, medications to reduce RBC production, and management of associated complications.

Blood platelets, also known as thrombocytes, are small, colorless cell fragments in our blood that play an essential role in normal blood clotting. They are formed in the bone marrow from large cells called megakaryocytes and circulate in the blood in an inactive state until they are needed to help stop bleeding. When a blood vessel is damaged, platelets become activated and change shape, releasing chemicals that attract more platelets to the site of injury. These activated platelets then stick together to form a plug, or clot, that seals the wound and prevents further blood loss. In addition to their role in clotting, platelets also help to promote healing by releasing growth factors that stimulate the growth of new tissue.

Refractory anemia is a type of anemia that does not respond to typical treatments, such as iron supplements or hormonal therapy. It is often associated with various bone marrow disorders, including myelodysplastic syndromes (MDS), a group of conditions characterized by abnormal blood cell production in the bone marrow.

In refractory anemia, the bone marrow fails to produce enough healthy red blood cells, leading to symptoms such as fatigue, weakness, shortness of breath, and pale skin. The condition can be difficult to treat, and treatment options may include more aggressive therapies such as immunosuppressive drugs, chemotherapy, or stem cell transplantation.

It is important to note that the term "refractory" in this context refers specifically to the lack of response to initial treatments, rather than a specific severity or type of anemia.

A mutation is a permanent change in the DNA sequence of an organism's genome. Mutations can occur spontaneously or be caused by environmental factors such as exposure to radiation, chemicals, or viruses. They may have various effects on the organism, ranging from benign to harmful, depending on where they occur and whether they alter the function of essential proteins. In some cases, mutations can increase an individual's susceptibility to certain diseases or disorders, while in others, they may confer a survival advantage. Mutations are the driving force behind evolution, as they introduce new genetic variability into populations, which can then be acted upon by natural selection.

Coagulants are substances that promote the process of coagulation or clotting. They are often used in medical settings to help control bleeding and promote healing. Coagulants work by encouraging the formation of a clot, which helps to stop the flow of blood from a wound or cut.

There are several different types of coagulants that may be used in medical treatments. Some coagulants are naturally occurring substances, such as vitamin K, which is essential for the production of certain clotting factors in the body. Other coagulants may be synthetic or semi-synthetic compounds, such as recombinant activated factor VII (rFVIIa), which is used to treat bleeding disorders and prevent excessive bleeding during surgery.

Coagulants are often administered through injection or infusion, but they can also be applied topically to wounds or cuts. In some cases, coagulants may be used in combination with other treatments, such as compression or cauterization, to help control bleeding and promote healing.

It is important to note that while coagulants can be helpful in controlling bleeding and promoting healing, they can also increase the risk of blood clots and other complications. As a result, they should only be used under the guidance and supervision of a qualified healthcare professional.

Thrombophilia is a medical condition characterized by an increased tendency to form blood clots (thrombi) due to various genetic or acquired abnormalities in the coagulation system. These abnormalities can lead to a hypercoagulable state, which can cause thrombosis in both veins and arteries. Commonly identified thrombophilias include factor V Leiden mutation, prothrombin G20210A mutation, antithrombin deficiency, protein C deficiency, and protein S deficiency.

Acquired thrombophilias can be caused by various factors such as antiphospholipid antibody syndrome (APS), malignancies, pregnancy, oral contraceptive use, hormone replacement therapy, and certain medical conditions like inflammatory bowel disease or nephrotic syndrome.

It is essential to diagnose thrombophilia accurately, as it may influence the management of venous thromboembolism (VTE) events and guide decisions regarding prophylactic anticoagulation in high-risk situations.

Chronic myeloid leukemia (CML), atypical, BCR-ABL negative is a rare subtype of CML that does not have the typical Philadelphia chromosome abnormality or the resulting BCR-ABL fusion gene. This means that the disease lacks the constitutively active tyrosine kinase that is targeted by imatinib mesylate (Gleevec) and other similar drugs.

The atypical form of CML is often characterized by a more aggressive clinical course, with a higher risk of transformation to acute leukemia compared to the classic form of CML. It can be difficult to diagnose and treat due to its rarity and heterogeneity. Treatment options may include chemotherapy, targeted therapy, stem cell transplantation, or a combination of these approaches. Regular follow-up with blood tests and bone marrow examinations is essential for monitoring the disease course and adjusting treatment as necessary.

An amino acid substitution is a type of mutation in which one amino acid in a protein is replaced by another. This occurs when there is a change in the DNA sequence that codes for a particular amino acid in a protein. The genetic code is redundant, meaning that most amino acids are encoded by more than one codon (a sequence of three nucleotides). As a result, a single base pair change in the DNA sequence may not necessarily lead to an amino acid substitution. However, if a change does occur, it can have a variety of effects on the protein's structure and function, depending on the nature of the substituted amino acids. Some substitutions may be harmless, while others may alter the protein's activity or stability, leading to disease.

Hematologic pregnancy complications refer to disorders related to the blood and blood-forming tissues that occur during pregnancy. These complications can have serious consequences for both the mother and the fetus if not properly managed. Some common hematologic pregnancy complications include:

1. Anemia: A condition characterized by a decrease in the number of red blood cells or hemoglobin in the blood, which can lead to fatigue, weakness, and shortness of breath. Iron-deficiency anemia is the most common type of anemia during pregnancy.
2. Thrombocytopenia: A condition characterized by a decrease in the number of platelets (cells that help blood clot) in the blood. Mild thrombocytopenia is relatively common during pregnancy, but severe thrombocytopenia can increase the risk of bleeding during delivery.
3. Gestational thrombotic thrombocytopenic purpura (GTTP): A rare but serious disorder that can cause blood clots to form in small blood vessels throughout the body, leading to a decrease in the number of platelets and red blood cells. GTTP can cause serious complications such as stroke, kidney failure, and even death if not promptly diagnosed and treated.
4. Disseminated intravascular coagulation (DIC): A condition characterized by abnormal clotting and bleeding throughout the body. DIC can be triggered by various conditions such as severe infections, pregnancy complications, or cancer.
5. Hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome: A serious complication of pregnancy that can cause damage to the liver and lead to bleeding. HELLP syndrome is often associated with preeclampsia, a condition characterized by high blood pressure and damage to organs such as the liver and kidneys.

It's important for pregnant women to receive regular prenatal care to monitor for these and other potential complications, and to seek prompt medical attention if any concerning symptoms arise.

Bone marrow is the spongy tissue found inside certain bones in the body, such as the hips, thighs, and vertebrae. It is responsible for producing blood-forming cells, including red blood cells, white blood cells, and platelets. There are two types of bone marrow: red marrow, which is involved in blood cell production, and yellow marrow, which contains fatty tissue.

Red bone marrow contains hematopoietic stem cells, which can differentiate into various types of blood cells. These stem cells continuously divide and mature to produce new blood cells that are released into the circulation. Red blood cells carry oxygen throughout the body, white blood cells help fight infections, and platelets play a crucial role in blood clotting.

Bone marrow also serves as a site for immune cell development and maturation. It contains various types of immune cells, such as lymphocytes, macrophages, and dendritic cells, which help protect the body against infections and diseases.

Abnormalities in bone marrow function can lead to several medical conditions, including anemia, leukopenia, thrombocytopenia, and various types of cancer, such as leukemia and multiple myeloma. Bone marrow aspiration and biopsy are common diagnostic procedures used to evaluate bone marrow health and function.

Platelet aggregation inhibitors are a class of medications that prevent platelets (small blood cells involved in clotting) from sticking together and forming a clot. These drugs work by interfering with the ability of platelets to adhere to each other and to the damaged vessel wall, thereby reducing the risk of thrombosis (blood clot formation).

Platelet aggregation inhibitors are often prescribed for people who have an increased risk of developing blood clots due to various medical conditions such as atrial fibrillation, coronary artery disease, peripheral artery disease, stroke, or a history of heart attack. They may also be used in patients undergoing certain medical procedures, such as angioplasty and stenting, to prevent blood clot formation in the stents.

Examples of platelet aggregation inhibitors include:

1. Aspirin: A nonsteroidal anti-inflammatory drug (NSAID) that irreversibly inhibits the enzyme cyclooxygenase, which is involved in platelet activation and aggregation.
2. Clopidogrel (Plavix): A P2Y12 receptor antagonist that selectively blocks ADP-induced platelet activation and aggregation.
3. Prasugrel (Effient): A third-generation thienopyridine P2Y12 receptor antagonist, similar to clopidogrel but with faster onset and greater potency.
4. Ticagrelor (Brilinta): A direct-acting P2Y12 receptor antagonist that does not require metabolic activation and has a reversible binding profile.
5. Dipyridamole (Persantine): An antiplatelet agent that inhibits platelet aggregation by increasing cyclic adenosine monophosphate (cAMP) levels in platelets, which leads to decreased platelet reactivity.
6. Iloprost (Ventavis): A prostacyclin analogue that inhibits platelet aggregation and causes vasodilation, often used in the treatment of pulmonary arterial hypertension.
7. Cilostazol (Pletal): A phosphodiesterase III inhibitor that increases cAMP levels in platelets, leading to decreased platelet activation and aggregation, as well as vasodilation.
8. Ticlopidine (Ticlid): An older P2Y12 receptor antagonist with a slower onset of action and more frequent side effects compared to clopidogrel or prasugrel.

Hemorrhage is defined in the medical context as an excessive loss of blood from the circulatory system, which can occur due to various reasons such as injury, surgery, or underlying health conditions that affect blood clotting or the integrity of blood vessels. The bleeding may be internal, external, visible, or concealed, and it can vary in severity from minor to life-threatening, depending on the location and extent of the bleeding. Hemorrhage is a serious medical emergency that requires immediate attention and treatment to prevent further blood loss, organ damage, and potential death.

The World Health Organization (WHO) is not a medical condition or term, but rather a specialized agency of the United Nations responsible for international public health. Here's a brief description:

The World Health Organization (WHO) is a specialized agency of the United Nations that acts as the global authority on public health issues. Established in 1948, WHO's primary role is to coordinate and collaborate with its member states to promote health, prevent diseases, and ensure universal access to healthcare services. WHO is headquartered in Geneva, Switzerland, and has regional offices around the world. It plays a crucial role in setting global health standards, monitoring disease outbreaks, and providing guidance on various public health concerns, including infectious diseases, non-communicable diseases, mental health, environmental health, and maternal, newborn, child, and adolescent health.

GPI-linked proteins are a type of cell surface protein that are attached to the plasma membrane via a glycosylphosphatidylinositol (GPI) anchor. The GPI anchor is a complex glycolipid molecule that acts as a molecular tether, connecting the protein to the outer leaflet of the lipid bilayer of the cell membrane.

The GPI anchor is synthesized in the endoplasmic reticulum (ER) and added to proteins in the ER or Golgi apparatus during protein trafficking. The addition of the GPI anchor to a protein occurs in a post-translational modification process called GPI anchoring, which involves the transfer of the GPI moiety from a lipid carrier to the carboxyl terminus of the protein.

GPI-linked proteins are found on the surface of many different types of cells, including red blood cells, immune cells, and nerve cells. They play important roles in various cellular processes, such as cell signaling, cell adhesion, and enzyme function. Some GPI-linked proteins also serve as receptors for bacterial toxins and viruses, making them potential targets for therapeutic intervention.

Thrombocytopenia is a medical condition characterized by an abnormally low platelet count (thrombocytes) in the blood. Platelets are small cell fragments that play a crucial role in blood clotting, helping to stop bleeding when a blood vessel is damaged. A healthy adult typically has a platelet count between 150,000 and 450,000 platelets per microliter of blood. Thrombocytopenia is usually diagnosed when the platelet count falls below 150,000 platelets/µL.

Thrombocytopenia can be classified into three main categories based on its underlying cause:

1. Immune thrombocytopenia (ITP): An autoimmune disorder where the immune system mistakenly attacks and destroys its own platelets, leading to a decreased platelet count. ITP can be further divided into primary or secondary forms, depending on whether it occurs alone or as a result of another medical condition or medication.
2. Decreased production: Thrombocytopenia can occur when there is insufficient production of platelets in the bone marrow due to various causes, such as viral infections, chemotherapy, radiation therapy, leukemia, aplastic anemia, or vitamin B12 or folate deficiency.
3. Increased destruction or consumption: Thrombocytopenia can also result from increased platelet destruction or consumption due to conditions like disseminated intravascular coagulation (DIC), thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), or severe bacterial infections.

Symptoms of thrombocytopenia may include easy bruising, prolonged bleeding from cuts, spontaneous nosebleeds, bleeding gums, blood in urine or stools, and skin rashes like petechiae (small red or purple spots) or purpura (larger patches). The severity of symptoms can vary depending on the degree of thrombocytopenia and the presence of any underlying conditions. Treatment for thrombocytopenia depends on the cause and may include medications, transfusions, or addressing the underlying condition.

A cavernous hemangioma is a type of benign vascular tumor that is made up of large, dilated blood vessels. It is characterized by the presence of large, "cavernous" spaces or sacs filled with blood. These lesions can occur in various parts of the body, but when they occur in the skin or mucous membranes, they appear as well-circumscribed rubbery masses that are compressible and blanchable (turn pale when pressed).

Cavernous hemangiomas are most commonly found on the face and neck, but they can also occur in other parts of the body such as the liver. They typically grow slowly during infancy or early childhood and then stabilize or even regress spontaneously over time. However, if they are located in critical areas such as the airway or near vital organs, they may require treatment to prevent complications.

Histologically, cavernous hemangiomas are composed of large, irregularly shaped vascular spaces lined by a single layer of endothelial cells and surrounded by fibrous tissue. Treatment options for cavernous hemangiomas include observation, compression therapy, laser therapy, surgical excision, or embolization.

Quinazolines are not a medical term per se, but they are a class of organic compounds that have been widely used in the development of various pharmaceutical drugs. Therefore, I will provide you with a chemical definition of quinazolines:

Quinazolines are heterocyclic aromatic organic compounds consisting of a benzene ring fused to a pyrazine ring. The structure can be represented as follows:

Quinazoline

They are often used as building blocks in the synthesis of various drugs, including those used for treating cancer, cardiovascular diseases, and microbial infections. Some examples of FDA-approved drugs containing a quinazoline core include the tyrosine kinase inhibitors gefitinib (Iressa) and erlotinib (Tarceva), which are used to treat non-small cell lung cancer, and the calcium channel blocker verapamil (Calan, Isoptin), which is used to treat hypertension and angina.

Cytokine receptors are specialized protein molecules found on the surface of cells that selectively bind to specific cytokines. Cytokines are signaling molecules used for communication between cells, and they play crucial roles in regulating immune responses, inflammation, hematopoiesis, and cell survival.

Cytokine receptors have specific binding sites that recognize and interact with the corresponding cytokines. This interaction triggers a series of intracellular signaling events that ultimately lead to changes in gene expression and various cellular responses. Cytokine receptors can be found on many different types of cells, including immune cells, endothelial cells, and structural cells like fibroblasts.

Cytokine receptors are typically composed of multiple subunits, which may include both extracellular and intracellular domains. The extracellular domain is responsible for cytokine binding, while the intracellular domain is involved in signal transduction. Cytokine receptors can be classified into several families based on their structural features and signaling mechanisms, such as the hematopoietic cytokine receptor family, the interferon receptor family, the tumor necrosis factor receptor family, and the interleukin-1 receptor family.

Dysregulation of cytokine receptors and their signaling pathways has been implicated in various diseases, including autoimmune disorders, chronic inflammation, and cancer. Therefore, understanding the biology of cytokine receptors is essential for developing targeted therapies to treat these conditions.

Isoantigens are antigens that are present on the cells or tissues of one individual of a species, but are absent or different in another individual of the same species. They are also known as "alloantigens." Isoantigens are most commonly found on the surface of red blood cells and other tissues, and they can stimulate an immune response when transplanted into a different individual. This is because the recipient's immune system recognizes the isoantigens as foreign and mounts a defense against them. Isoantigens are important in the field of transplantation medicine, as they must be carefully matched between donor and recipient to reduce the risk of rejection.

Most people with essential thrombocythemia are without symptoms at the time of diagnosis, which is usually made after noting an ... Essential thrombocythemia can be linked with a three-fold increase in risk of miscarriage. Throughout pregnancy, close ... Essential thrombocythemia is sometimes described as a slowly progressive disorder with long asymptomatic periods punctuated by ... "Essential Thrombocythemia". The Lecturio Medical Concept Library. Retrieved 22 July 2021. Valera, MC; Parant, O; Vayssiere, C; ...
... the term thrombocythemia is used, as either primary thrombocythemia or essential thrombocythemia. The condition arises from a ... The most common cause of clonal thrombocythemia is a myeloproliferative neoplasm. These include: essential thrombocythemia, ... However, in essential thrombocythemia where platelet counts are over 750x109/L or 1,000x109/L, especially if there are other ... Reactive thrombocythemia is the most common cause of a high platelet count. It accounts for 88% to 97% of thrombocythemia cases ...
Vannucchi AM, Guglielmelli P, Pieri L, Antonioli E, Bosi A (February 2009). "Treatment options for essential thrombocythemia ...
... , also known as hydroxyurea, is a medication used in sickle-cell disease, essential thrombocythemia, chronic ... July 2005). "Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia". The New England Journal of Medicine ... Hydroxycarbamide is used for the following indications: Myeloproliferative disease (primarily essential thrombocythemia and ... It is on the World Health Organization's List of Essential Medicines. Hydroxycarbamide is available as a generic medication. ...
... is a drug used for the treatment of essential thrombocytosis (also known as essential thrombocythemia), or overproduction of ... July 2005). "Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia". The New England Journal of Medicine ... Brière JB (January 2007). "Essential thrombocythemia". Orphanet Journal of Rare Diseases. 2 (1): 3. doi:10.1186/1750-1172-2-3. ... Reilly JT (February 2009). "Anagrelide for the treatment of essential thrombocythemia: a survey among European hematologists/ ...
Reikvam H, Tiu RV (April 2012). "Venous thromboembolism in patients with essential thrombocythemia and polycythemia vera". ... essential thrombocythemia, intravenous drug use, and smoking. Some risk factors influence the location of DVT within the body. ...
December 1988). "[Therapeutic effect of ranimustine (MCNU) on essential thrombocythemia and polycythemia vera]". Gan To Kagaku ...
Passamonti F, Lazzarino M (September 2003). "Treatment of polycythemia vera and essential thrombocythemia: the role of ...
A classic symptom of polycythemia vera (and the related myeloproliferative disease essential thrombocythemia) is ... essential thrombocythemia, and myeloid metaplasia with myelofibrosis". Cancer Cell. 7 (4): 387-97. doi:10.1016/j.ccr.2005.03. ... a pathognomonic microvascular thrombotic complication in essential thrombocythemia and polycythemia vera". Semin Thromb Hemost ...
Specific mutations to this gene are associated with myelofibrosis and essential thrombocythemia. In essential thrombocythemia, ...
2002). "Overexpression of the polycythemia rubra vera-1 gene in essential thrombocythemia". J. Clin. Oncol. 20 (20): 4249-54. ... association between elevated PRV-1 mRNA expression and low plasma erythropoietin concentration in essential thrombocythaemia". ...
Fibrosis identified in the bone marrow biopsies of patients with essential thrombocythemia:[..] (2008) Cesk. Patol. Vol. 44 No ...
... platelets are both elevated and activated Essential thrombocythemia Polycythemia vera Associated with other myeloid neoplasms ... "Acquired von Willebrand's disease in association with essential thrombocythemia: regression following treatment". Acta ... Calcium ions are essential for the binding of these coagulation factors. In addition to interacting with vWF and fibrin, ...
Essential thrombocythemia (ET) is a disorder characterized by elevated numbers of circulating platelets. The disease occurs in ... major thrombosis and bleeding complications in essential thrombocythaemia and polycythaemia vera". Platelets. 15 (2): 67-84. ... It is essential for the formation of an adequate quantity of platelets. After budding off platelets, what remains is mainly the ... "Megakaryocyte quantifications in relation to thrombokinetics in primary thrombocythaemia and allied diseases". Scand. J. ...
May 2009). "TET2 mutations and their clinical correlates in polycythemia vera, essential thrombocythemia and myelofibrosis". ...
"Bioinformatics Analysis of Key Genes and Pathways Associated with Thrombosis in Essential Thrombocythemia". Medical Science ...
"Bioinformatics Analysis of Key Genes and Pathways Associated with Thrombosis in Essential Thrombocythemia". Medical Science ... "Zebrafish peptidoglycan recognition proteins are bactericidal amidases essential for defense against bacterial infections". ...
A third Imago-sponsored global clinical trial for the treatment of essential thrombocythemia was begun in 2020. The results of ... "Study of Bomedemstat in Participants With Essential Thrombocythemia (IMG-7289-CTP-201/MK-3543-003)" at ClinicalTrials.gov ... under development by Imago BioSciences for the treatment of myeloproliferative neoplasms including essential thrombocythemia, ... a co-factor essential for the oxidative demethylation reaction. The first step in the catalytic reaction of LSD1 involves the ...
... in essential thrombocythemia: quantitative and qualitative abnormalities of bone marrow CFU-Meg". Am. J. Hematol. 24 (1): 23-30 ...
Givinostat (ITF2357) is also under investigation for treatment of polycythemia vera (PV), essential thrombocythemia (ET) and ...
Mutations in JAK2 have been implicated in polycythemia vera, essential thrombocythemia, and myelofibrosis as well as other ... Neubauer H, Cumano A, Müller M, Wu H, Huffstadt U, Pfeffer K (May 1998). "Jak2 deficiency defines an essential developmental ...
"Development of acute myocardial infarction in a young female patient with essential thrombocythemia treated with anagrelide: a ...
... and c-MPL genes detected by fluorescence in situ hybridization in essential thrombocythemia". Cancer Genetics and Cytogenetics ...
... essential thrombocythemia. In these cases, myelofibrosis occurs as a result of somatic evolution of the abnormal hematopoietic ... specifically polycythemia vera and essential thrombocythemia. The JAK2 protein is mutated giving risk to a variant protein with ... post-polycythemia vera and post-essential thrombocythemia], in adults with anemia. Myelofibrosis was first described in 1879 by ... post-polycythemia vera or post-essential thrombocythemia) myelofibrosis (MF). In March 2022, the FDA approved pacritinib (Vonjo ...
They are also being studied in psoriasis, polycythemia vera, alopecia, essential thrombocythemia, ulcerative colitis, myeloid ... JH2 is a "pseudokinase domain", a domain structurally similar to a tyrosine kinase and essential for a normal kinase activity, ...
A phase II trial is underway for patients with myeloproliferative neoplasms, polycythemia vera, essential thrombocythemia, or ...
... or Post-polycythemia Vera or Post-essential Thrombocythemia Myelofibrosis (Post-PV/ET MF)". ClinicalTrials.gov. Archived from ...
Symptoms and presentation can mimic essential thrombocythemia, with the main differentiator for pre-PMF being the presence of ... Both pre-PMF and Essential thrombocythemia can share diagnostic similarities, such as a proliferation of megakaryocytes and a ...
Essential thrombocythemia (ET) is diagnosed with a platelet count greater than 450 × 109/L and is associated with the JAK2 ... December 2005). "Definition of subtypes of essential thrombocythaemia and relation to polycythaemia vera based on JAK2 V617F ... Overt Fibrotic Stage Essential thrombocythemia (ET) Chronic eosinophilic leukemia (not otherwise specified) MPN, unclassifiable ... with a publication bias suspected for essential thrombocythemia and primary myelofibrosis. The concept of myeloproliferative ...
... in patients with polycythaemia vera or essential thrombocythaemia with JAK2-V617F mutation". British Journal of Haematology. ... These include a multi-center Phase II trial involving 37 patients with V617F JAK2 positive polycythemia vera and essential ... V617F JAK2 positive polycythemia vera and essential thrombocytosis, and refractory neuroblastoma. The most significant effort ...
Most people with essential thrombocythemia are without symptoms at the time of diagnosis, which is usually made after noting an ... Essential thrombocythemia can be linked with a three-fold increase in risk of miscarriage. Throughout pregnancy, close ... Essential thrombocythemia is sometimes described as a slowly progressive disorder with long asymptomatic periods punctuated by ... "Essential Thrombocythemia". The Lecturio Medical Concept Library. Retrieved 22 July 2021. Valera, MC; Parant, O; Vayssiere, C; ...
... thrombocythemia). Explore symptoms, inheritance, genetics of this condition. ... Essential thrombocythemia is a condition characterized by an increased number of platelets ( ... medlineplus.gov/genetics/condition/essential-thrombocythemia/ Essential thrombocythemia. ... Essential thrombocythemia is a condition characterized by an increased number of platelets (thrombocythemia). Platelets ( ...
Experts discuss essential thrombocythemia, including the role of CALR mutations, cytoreductive therapy, and JAK inhibition, ... Treatment Considerations for Patients With Essential Thrombocythemia: A Conversation Between Experts. Clinical Thought. ... Two experts discuss their approach to managing patients with essential thrombocythemia, including the role of CALR mutations, ...
... also known as primary thrombocythemia, is a rare blood disease characterized by an overproduction of platelets in the bone ... or feet in cases of essential thrombocythemia.. Essential thrombocythemia manifests through signs and symptoms that arise as a ... Essential thrombocythemia, also known as primary thrombocythemia, is a rare blood disease characterized by an overproduction of ... and resulting in essential thrombocythemia.. Risk factors. The following factors increase the risk in developing essential ...
Ropeginterferon alfa-2b LAis under clinical development by PharmaEssentia and currently in Phase III for Essential ... Ropeginterferon alfa-2b LA by PharmaEssentia for Essential Thrombocythemia: Likelihood of Approval. Brought to you by ... According to GlobalData, Phase III drugs for Essential Thrombocythemia does not have sufficient historical data to build an ... essential thrombocythemia, chronic myeloid leukaemia and corona virus caused by SARS(COVID-19). The drug candidate is ...
Most women who have essential thrombocythemia have normal, healthy pregnancies. But uncontrolled thrombocythemia can lead to ... Essential thrombocythemia is a chronic disease with no cure. If you have a mild form of the disease, you may not need treatment ... Essential thrombocythemia. This disorder causes your body to make too many platelets, a sticky component of blood cells. Having ... Essential thrombocythemia (throm-boe-sie-THEE-me-uh) is an uncommon disorder in which your body produces too many platelets. ...
Essential Thrombocythemia. Essential Thrombocythemia (ET) or Primary Thrombocythemia, is a rare, chronic myeloproliferative ... Read More about Essential Thrombocythemia. ET is characterized by a persisting elevated platelet count ,450,000/µL, with ...
Essential thrombocythemia is a disease in which too many platelets are in the blood. This abnormal increase is due to increased ... Essential thrombocythemia is a disease in which too many platelets (thrombocytes) in the blood. This abnormal increase in the ...
Essential Thrombocythemia. Essential thrombocythemia results when the bone marrow produces too many platelets. The condition ... Some people with essential thrombocythemia develop myelofibrosis . We are exploring new treatment options for essential ... To make a diagnosis of essential thrombocythemia, our doctors usually do a bone marrow biopsy. In this test, a needle is ... thrombocythemia and for people who develop myelofibrosis after essential thrombocythemia and are not responding to standard ...
... is the commonest MPN, and is predominantly characterised by ...
When this process occurs without other blood cell disorders, its called essential thrombocythemia. ... Thrombocythemia and Thrombocytosis - What Causes Thrombocythemia and Thrombocytosis? - Causes Primary Thrombocythemia. In this ... In addition to the bone marrow making too many platelets, the platelets also are abnormal in primary thrombocythemia. They may ... A rare form of thrombocythemia is inherited. ("Inherited" means the condition is passed from parents to children through the ...
A patient shares their experience with essential thrombocythemia ... Patient Experience - Essential Thrombocythemia. By William Aird ...
How to Treat Essential Thrombocythemia in Ayurveda. ABSTRACT. Essential Tthrombocythemia is a rare disorder which is ... Essential thrombocythemia is an uncommon disorder which is caused due to abnormal mutation in certain genes. In this platelet ... Essential thrombocythemia is known as a type of chronic myeloproliferative disorder because in this the spongy tissue inside ... As the essential thrombocythemia is considered to be an incurable disorder, hence the treatment is aimed at controlling the ...
Essential thrombocythemia (ET) Polycythemia vera (PV) Primary myelofibrosis (PMF) Driver mutation distributions… ... essential thrombocythemia (n=206) or (B) polycythemia vera (n=55). ET: essential thrombocythemia; PV: polycythemia vera; 3NEG: ... Current treatment algorithm in essential thrombocythemia. , Tefferi, A., Vannucchi, A. M., & Barbui, T. (2018). Essential ... A, and B, Bone marrow megakaryocytic clusters in essential thrombocythemia. , Tefferi A. Thrombocytosis. In: Michelson A, ...
... is called thrombocythemia. Three types of blood cells are available namely red blood cells, white blood cells and platelets. ... Essential thrombocythemia is common in old age and is more prevalent in women than men. ... Thrombocythemia. By surekha. The condition in which excess of blood platelets called thrombocytes are produced, is called ... Out of these, the cookies that are categorized as necessary are stored on your browser as they are essential for the working of ...
Essential Thrombocythemia - Learn about the causes, symptoms, diagnosis & treatment from the MSD Manuals - Medical Consumer ... To distinguish primary thrombocythemia from secondary thrombocythemia Secondary Thrombocythemia Secondary thrombocythemia is ... Essential Thrombocythemia: Supports research as well as advocacy and education for people with essential thrombocythemia ... Essential Thrombocythemia (Primary Thrombocythemia). By Jane Liesveld , MD, James P. Wilmot Cancer Institute, University of ...
Thrombocythemia, Essential. Vital. It is feasible that the major title of the record Essential Thrombocythemia is not the name ... Vital thrombocythemia, additionally called ET, is an unusual illness. One of the most essential initial truth concerning ET: ...
Essential; Thrombocythemia, Hemorrhagic; Thrombocythemia, Idiopathic; Thrombocythemia, Primary; Hemorrhagic Thrombocythemia. On ... Thrombocythemia, Essential; Thrombocythemia, Hemorrhagic; Thrombocythemia, Idiopathic; Thrombocythemia, Primary; Hemorrhagic ...
Read about being diagnosed with a myeloproliferative neoplasm (MPN) from people who have been there. Find information on living with an MPN and finding an expert.
The goal of therapy for essential thrombocythemia (ET) and polycythemia vera (PV) patients is to reduce thrombotic events by ... A Randomized, Phase 3, Trial of Interferon-α versus Hydroxyurea in Polycythemia Vera and Essential Thrombocythemia. ... The goal of therapy for essential thrombocythemia (ET) and polycythemia vera (PV) patients is to reduce thrombotic events by ... A Randomized, Phase 3, Trial of Interferon-α versus Hydroxyurea in Polycythemia Vera and Essential Thrombocythemia. ...
Cytogenetic abnormalities in essential thrombocythemia at presentation and transformation. International Journal of Hematology ... Cytogenetic abnormalities in essential thrombocythemia at presentation and transformation. Matjaz Sever, Hagop Kantarjian, ... Cytogenetic abnormalities in essential thrombocythemia at presentation and transformation. In: International Journal of ... Cytogenetic abnormalities in essential thrombocythemia at presentation and transformation. / Sever, Matjaz; Kantarjian, Hagop; ...
Thrombocythemia Research Paper. Essential Thromboycythemia, also sometimes referred to as idiopathic thrombocythemia, essential ... Symptoms of essential thrombocythemia are largely based on where a clot may have formed in the body. Some symptoms include but ... Blood is essential to human life. It carries oxygen, nutrients and hormones all through your body with a strong pump from the ... There are two types of hemophilia, A and B (Christmas Disease). Low levels or complete absence of a blood protein essential for ...
Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation ... prefibrotic primary myelofibrosis (pre-PMF) / essential thrombocythemia (ET) / differentialdiagnosis / predictive model. Source ... Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation ... "Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation ...
Using a JAK2 knock-in mouse model of essential thrombocythemia, Hobbs et al. showed that the proportion of JAK2-positive ... Reticulin accumulation in essential thrombocythemia: prognostic significance and relationship to therapy. J Clin Oncol. 2009; ... Essential thrombocythemia/polycythemia vera. Increased reticulin BMF has been reported in approximately 20% of patients with ... studied patients with MPN and reported that proplatelet numbers were higher in patients with essential thrombocythemia and pre- ...
Essential thrombocythemia with enlarged spleen 4. Hello. I have essential thrombocythemia ( a bone marrow chronic disorder with ...
IMAGO BIOSCIENCES GRANTED FAST TRACK DESIGNATION FOR IMG-7289 IN ESSENTIAL THROMBOCYTHEMIA. ... for the treatment of Essential Thrombocythemia. This follows the Fast Track designation for the same drug in Myelofibrosis in ... essential thrombocythemia, and myelofibrosis, known collectively as myeloproliferative neoplasms (MPNs).. MPN Research ...
If you click Reject all non-essential cookies only necessary cookies providing core functionality such as security, network ...
Essential thrombocythemia (ET) is a disorder of the bone marrow characterized by a neoplastic overproduction of platelets ( ... High platelet counts in essential thrombocythemia (ET) can be effectively lowered by treatment with either anagrelide or ... Non-inferiority of anagrelide compared to hydroxyurea in WHO-essential ET. (Prepublished online, Blood, Jan. 11, 2013). ...
In a multicenter European retrospective study including 162 patients with COVID-19 occurring in essential thrombocythemia (ET, ... In a multicenter European retrospective study including 162 patients with COVID-19 occurring in essential thrombocythemia (ET, ... Among classic myeloproliferative neoplasms, essential thrombocythemia is associated with the greatest risk of venous ...
Essential thrombocythemia has an incidence of 0.1-1.5 per 100,000 per year. Myelofibrosis has an international incidence of 0.4 ... True incidences of essential thrombocythemia and myelofibrosis are not known because epidemiological studies on these disorders ... PCR assay run on bone marrow: To test for JAK2; available for suspected cases of polycythemia vera, essential thrombocythemia, ... In polycythemia vera, essential thrombocythemia, and myelofibrosis (see the following images), the prevalent genetic lesion ...
  • In 2013, two groups detected calreticulin mutations in a majority of JAK2-negative/MPL-negative patients with essential thrombocythemia and primary myelofibrosis, which makes CALR mutations the second most common in myeloproliferative neoplasms. (wikipedia.org)
  • Ropeginterferon alpha-2b (P-1101/AOP2014) is under development for the treatment of hepatitis B, adult T-cell leukemia, hepatitis C and hepatitis D infections, polycythemia vera, primary myelofibrosis, essential thrombocythemia, chronic myeloid leukaemia and corona virus caused by SARS(COVID-19). (pharmaceutical-technology.com)
  • Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation with thrombocytosis. (ac.rs)
  • TY - JOUR AU - Leković, Danijela AU - Bogdanović, Andrija AU - Sobas, Marta AU - Arsenović, Isidora AU - Smiljanić, Mihailo AU - Ivanović, Jelena AU - Bodrožić, Jelena AU - ÄŒokić, Vladan AU - Milić, NataÅ¡a PY - 2023 UR - http://rimi.imi.bg.ac.rs/handle/123456789/1344 AB - Essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (prePMF) initially have a similar phenotypic presentation with thrombocytosis. (ac.rs)
  • MF can present as primary myelofibrosis (PMF), or arise from a pre-existing diagnosis of polycythemia vera or essential thrombocythemia. (haematologica.org)
  • MPN Research Foundation has a single goal: to stimulate original research in pursuit of new treatments - and eventually a cure - for polycythemia vera, essential thrombocythemia, and myelofibrosis, known collectively as myeloproliferative neoplasms (MPNs). (mpnresearchfoundation.org)
  • The 2016 World Health Organization (WHO) diagnostic criteria for myeloproliferative neoplasms (MPN) underscore the prognostically-relevant distinction between essential thrombocythemia (ET) and prefibrotic primary myelofibrosis (pre-PMF). (elsevierpure.com)
  • Post- essential thrombocythemia myelofibrosis. (cancer.gov)
  • The classic myeloproliferative neoplasms, including chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are a phenotypically diverse category of malignancies that are derived from stem cells in the myeloid lineage. (mhmedical.com)
  • the former constitutes the topic of this chapter and includes chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). (mhmedical.com)
  • 2005). In addition, about half of patients with the closely related blood diseases, essential thrombocythemia (ET) and primary myelofibrosis (PMF), also carry the JAK21 m utation (Baxter et al. (cdc.gov)
  • This is often found in essential thrombocythaemia, polycythemia vera and primary myelofibrosis. (edu.au)
  • On 26 November 2010, orphan designation (EU/3/10/810) was granted by the European Commission to Dr Ulrich Granzer, Germany, for N-tert-butyl-3-[(5-methyl-2-{[4-(2-pyrrolidin-1-ylethoxy)phenyl]amino}pyrimidin-4-yl)amino] benzenesulfonamide dihydrochloride monohydrate for the treatment of post-essential thrombocythaemia myelofibrosis. (europa.eu)
  • N-tert-butyl-3-[(5-methyl-2-{[4-(2-pyrrolidin-1-ylethoxy)phenyl]amino}pyrimidin-4-yl)amino] benzenesulfonamide dihydrochloride monohydrate for treatment of post-essential thrombocythaemia myelofibrosis has been authorised in the EU as Inrebic since 8 February 2021. (europa.eu)
  • What is post-essential thrombocythaemia myelofibrosis? (europa.eu)
  • Post-essential thrombocythaemia myelofibrosis is a debilitating disease that is long-lasting and may be life-threatening because it can lead to severe anaemia (low red-blood-cell counts) and infections, and can result in leukaemia (cancer of the white blood cells). (europa.eu)
  • At the time of designation, post-essential thrombocythaemia myelofibrosis affected less than 0.15 in 10,000 people in the European Union (EU). (europa.eu)
  • At the time of designation, although hydroxyurea and busulfan were authorised in the EU for primary myelofibrosis (myelofibrosis of unknown cause), there were no treatments authorised specifically for post-essential thrombocythaemia myelofibrosis. (europa.eu)
  • At the time of submission of the application for orphan designation , clinical trials with the medicine including patients with post-essential thrombocythaemia myelofibrosis were ongoing. (europa.eu)
  • At the time of submission, this medicine was not authorised anywhere in the EU for post-essential thrombocythaemia myelofibrosis. (europa.eu)
  • Subtypes include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). (researchgate.net)
  • Philadelphia-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). (researchgate.net)
  • JAK1/JAK2 kinase inhibitor indicated for treatment of patients with intermediate or high-risk myelofibrosis, including primary myelofibrosis, post-polycythemia vera myelofibrosis and post-essential thrombocythemia myelofibrosis. (medscape.com)
  • In hematology, essential thrombocythemia (ET) is a rare chronic blood cancer (myeloproliferative neoplasm) characterised by the overproduction of platelets (thrombocytes) by megakaryocytes in the bone marrow. (wikipedia.org)
  • Essential thrombocythemia is a condition characterized by an increased number of platelets (thrombocythemia). (medlineplus.gov)
  • Another problem in essential thrombocythemia is abnormal bleeding, which occurs more often in people with a very high number of platelets. (medlineplus.gov)
  • Excess platelets can cause thrombosis, which leads to many signs and symptoms of essential thrombocythemia. (medlineplus.gov)
  • Essential thrombocythemia, also known as primary thrombocythemia, is a rare blood disease characterized by an overproduction of platelets in the bone marrow. (vejthani.com)
  • Symptoms of essential thrombocythemia can vary among individuals as it progresses gradually, leading to increased production of platelets in the bone marrow and elevated platelet levels. (vejthani.com)
  • Essential thrombocythemia (throm-boe-sie-THEE-me-uh) is an uncommon disorder in which your body produces too many platelets. (ahdubai.com)
  • Less commonly, essential thrombocythemia may cause bleeding, especially if your platelet count is more than 1 million platelets per microliter of blood. (ahdubai.com)
  • In the case of essential thrombocythemia, the bone marrow makes too many cells that create platelets. (ahdubai.com)
  • Essential Thrombocythemia (ET) or Primary Thrombocythemia, is a rare, chronic myeloproliferative disorder that involves the over production of blood platelets by the marrow. (jdforecasting.com)
  • Essential thrombocythemia is a disease in which too many platelets (thrombocytes) in the blood. (mnoncology.com)
  • In addition to the bone marrow making too many platelets, the platelets also are abnormal in primary thrombocythemia. (nih.gov)
  • Although the platelet count is high in secondary thrombocytosis, the platelets are normal (unlike in primary thrombocythemia). (nih.gov)
  • In thrombocythemia, megakaryocytes increase in number and produce too many platelets. (msdmanuals.com)
  • Secondary Thrombocythemia Secondary thrombocythemia is excess platelets in the bloodstream that develops as a result of another disorder and rarely leads to excessive blood clotting or bleeding. (msdmanuals.com)
  • I have essential thrombocythemia ( a bone marrow chronic disorder with overproduction of platelets) and with mildly enlarged spleen. (abchomeopathy.com)
  • Essential thrombocythemia (ET) is a disorder of the bone marrow characterized by a neoplastic overproduction of platelets (thrombocytes). (aop-health.com)
  • It can develop as a reaction to essential thrombocythaemia (overproduction of platelets, components that help the blood to clot). (europa.eu)
  • Essential thrombocythemia or excess production of platelets. (apollohospitals.com)
  • Compared with essential thrombocythemia, secondary thrombocytosis causes less risk of blood clots and bleeding. (ahdubai.com)
  • Essential thrombocythaemia (ET) is the commonest MPN, and is predominantly characterised by excessive clonal megakaryocyte production and thrombocytosis. (streamliners.co.nz)
  • Essential thrombocythemia is characterized by proliferation of hematopoietic tissue predominantly involving megakaryocytes and resulting in marked thrombocytosis. (ashpublications.org)
  • Essential thrombocythemia is one of the 'classic' Philadelphia chromosome negative myeloproliferative neoplasm characterized by sustained thrombocytosis, increased megakaryopoiesis and high risk of vascular complications. (isth.org)
  • A Randomized, Phase 3, Trial of Interferon-α versus Hydroxyurea in Polycythemia Vera and Essential Thrombocythemia. (ox.ac.uk)
  • Statin use, survival and incidence of thrombosis among older patients with polycythemia vera and essential thrombocythemia. (bvsalud.org)
  • Primary thrombocythemia may or may not cause symptoms. (msdmanuals.com)
  • A, and B, Bone marrow megakaryocytic clusters in essential thrombocythemia. (capsulehealth.one)
  • Essential thrombocythemia is a type of chronic myeloproliferative disorder. (ahdubai.com)
  • Essential thrombocythemia manifests through signs and symptoms that arise as a result of elevated platelet counts, leading to the formation of blood clots (thrombi). (vejthani.com)
  • He or she will rule out all other causes of high platelet counts to confirm a diagnosis of essential thrombocythemia. (ahdubai.com)
  • High platelet counts in essential thrombocythemia (ET) can be effectively lowered by treatment with either anagrelide or hydroxyurea. (aop-health.com)
  • Abnormal blood clotting (thrombosis) is common in people with essential thrombocythemia and causes many signs and symptoms of this condition. (medlineplus.gov)
  • Essential thrombocythemia is sometimes described as a slowly progressive disorder with long asymptomatic periods punctuated by thrombotic or hemorrhagic events. (wikipedia.org)
  • Essential thrombocythemia is a genetic disorder that is acquired rather than being present at birth. (vejthani.com)
  • Mutations in the JAK2 , MPL , and THPO genes that are associated with essential thrombocythemia lead to overactivation of the JAK/STAT pathway. (medlineplus.gov)
  • Although mutations in the CALR and TET2 genes have been found in people with essential thrombocythemia, it is unclear how these gene mutations are involved in development of the condition. (medlineplus.gov)
  • Two experts discuss their approach to managing patients with essential thrombocythemia, including the role of CALR mutations, cytoreductive therapy, and JAK inhibition. (clinicaloptions.com)
  • Essential thrombocythemia is considered an acquired genetic condition, meaning it occurs when specific genes undergo mutations or changes. (vejthani.com)
  • In summary, the mutations in these genes lead to an abnormal increase in platelet production, exceeding the body's needs, and resulting in essential thrombocythemia. (vejthani.com)
  • Driver mutations among very young patients with (A) essential thrombocythemia (n=206) or (B) polycythemia vera (n=55). (capsulehealth.one)
  • The JAK2 and CALR genes are the most commonly mutated genes in essential thrombocythemia. (medlineplus.gov)
  • You may not have any noticeable symptoms of essential thrombocythemia. (ahdubai.com)
  • Most people with essential thrombocythemia are without symptoms at the time of diagnosis, which is usually made after noting an elevated platelet level on a routine complete blood count (CBC). (wikipedia.org)
  • The diagnosis of essential thrombocythemia was considered to be firm in two patients and probable in the third one. (ashpublications.org)
  • The main objective of treating essential thrombocythemia is to minimize the likelihood of thrombotic events. (vejthani.com)
  • When this process occurs without other blood cell disorders, it's called essential thrombocythemia. (nih.gov)
  • Essential thrombocythemia usually occurs in people over age 50. (msdmanuals.com)
  • Some people with essential thrombocythemia have episodes of severe pain, redness, and swelling ( erythromelalgia ), which commonly occur in the hands and feet. (medlineplus.gov)
  • Medications aimed at preventing blood clots and reducing platelet levels are commonly prescribed by healthcare providers to manage essential thrombocythemia. (vejthani.com)
  • Less commonly, essential thrombocythemia can cause clots in the arteries that supply blood to your heart. (ahdubai.com)
  • Characteristics and outcomes of patients with essential thrombocythemia or polycythemia vera diagnosed before 20 years of age: a systematic review. (capsulehealth.one)
  • The goal of therapy for essential thrombocythemia (ET) and polycythemia vera (PV) patients is to reduce thrombotic events by normalizing blood counts. (ox.ac.uk)
  • Cytogenetic abnormalities in patients with essential thrombocythemia (ET) are infrequent. (elsevierpure.com)
  • Reduces elevated platelet count in patients with essential thrombocythemia and polycythemia vera. (medscape.com)
  • It has been argued that essential thrombocythemia should be classified together with those disorders as a myeloproliferative syndrome. (ashpublications.org)
  • Imago BioSciences announced that the U.S. Food and Drug Administration (FDA) has granted Fast Track designation for the development of IMG-7289 (bomedemstat) for the treatment of Essential Thrombocythemia. (mpnresearchfoundation.org)
  • While blood clots can develop anywhere in the body, they tend to occur more frequently in the brain, hands, or feet in cases of essential thrombocythemia. (vejthani.com)
  • Clots can develop anywhere in your body, but with essential thrombocythemia they occur most often in your brain, hands and feet. (ahdubai.com)
  • Essential thrombocythemia is a chronic disease with no cure. (ahdubai.com)
  • Some people with essential thrombocythemia do not have a mutation in any of the known genes associated with this condition. (medlineplus.gov)
  • AML transformation in essential thrombocythemia is considered relatively rare. (ajmc.com)
  • Non-inferiority of anagrelide compared to hydroxyurea in WHO-essential ET. (aop-health.com)
  • Although essential thrombocythemia is not curable, appropriate treatment strategies can help reduce the risk of developing serious complications. (vejthani.com)
  • Current treatment algorithm in essential thrombocythemia. (capsulehealth.one)
  • Essential thrombocythemia treatment algorithm 2018. (capsulehealth.one)
  • A rare form of thrombocythemia is inherited. (nih.gov)
  • Essential thrombocythemia can lead to a variety of potentially life-threatening complications. (ahdubai.com)
  • But uncontrolled thrombocythemia can lead to miscarriage and other complications. (ahdubai.com)
  • Essential thrombocythemia affects an estimated 1 to 24 per 1 million people worldwide. (medlineplus.gov)
  • Essential thrombocythemia is more common in people over age 60, though younger people can develop it too. (ahdubai.com)
  • The following factors increase the risk in developing essential thrombocythemia. (vejthani.com)
  • Polycythemia vera (PV) and essential thrombocythemia (ET) are linked to increased risk of cardiovascular morbidity and mortality . (bvsalud.org)
  • Most cases of essential thrombocythemia are not inherited. (medlineplus.gov)
  • In some cases, individuals with essential thrombocythemia (ET) and an exceptionally elevated platelet count may experience bleeding as a result of the condition. (vejthani.com)
  • Essential' means that the thrombocythaemia is not caused by any known condition. (europa.eu)
  • When it is inherited, the condition is called familial essential thrombocythemia. (medlineplus.gov)