A heterogeneous group of disorders with the common feature of prolonged eosinophilia of unknown cause and associated organ system dysfunction, including the heart, central nervous system, kidneys, lungs, gastrointestinal tract, and skin. There is a massive increase in the number of EOSINOPHILS in the blood, mimicking leukemia, and extensive eosinophilic infiltration of the various organs.
Abnormal increase of EOSINOPHILS in the blood, tissues or organs.
Granular leukocytes with a nucleus that usually has two lobes connected by a slender thread of chromatin, and cytoplasm containing coarse, round granules that are uniform in size and stainable by eosin.
A PDGF receptor that binds specifically to both PDGF-A chains and PDGF-B chains. It contains a protein-tyrosine kinase activity that is involved in SIGNAL TRANSDUCTION.
Factors that are involved in directing the cleavage and POLYADENYLATION of the of MESSENGER RNA near the site of the RNA 3' POLYADENYLATION SIGNALS.
A characteristic symptom complex.
A cytokine that promotes differentiation and activation of EOSINOPHILS. It also triggers activated B-LYMPHOCYTES to differentiate into IMMUNOGLOBULIN-secreting cells.
Proteins found in EOSINOPHIL granules. They are primarily basic proteins that play a role in host defense and the proinflammatory actions of activated eosinophils.
Widespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from POLYARTERITIS NODOSA.
BENZOIC ACID amides.
A CC-type chemokine that is found at high levels in the THYMUS and has specificity for CCR4 RECEPTORS. It is synthesized by DENDRITIC CELLS; ENDOTHELIAL CELLS; KERATINOCYTES; and FIBROBLASTS.
A condition characterized by the thickening of the ventricular ENDOCARDIUM and subendocardium (MYOCARDIUM), seen mostly in children and young adults in the TROPICAL CLIMATE. The fibrous tissue extends from the apex toward and often involves the HEART VALVES causing restrictive blood flow into the respective ventricles (CARDIOMYOPATHY, RESTRICTIVE).
Piperazines are a class of heterocyclic organic compounds containing a seven-membered ring with two nitrogen atoms at positions 1 and 4, often used in pharmaceuticals as smooth muscle relaxants, antipsychotics, antidepressants, and antihistamines, but can also be found as recreational drugs with stimulant and entactogen properties.
A family of 6-membered heterocyclic compounds occurring in nature in a wide variety of forms. They include several nucleic acid constituents (CYTOSINE; THYMINE; and URACIL) and form the basic structure of the barbiturates.
A heterogenous group of disorders characterized by the abnormal increase of MAST CELLS in only the skin (MASTOCYTOSIS, CUTANEOUS), in extracutaneous tissues involving multiple organs (MASTOCYTOSIS, SYSTEMIC), or in solid tumors (MASTOCYTOMA).
A condition characterized by infiltration of the lung with EOSINOPHILS due to inflammation or other disease processes. Major eosinophilic lung diseases are the eosinophilic pneumonias caused by infections, allergens, or toxic agents.
A group of disorders caused by the abnormal proliferation of MAST CELLS in a variety of extracutaneous tissues including bone marrow, liver, spleen, lymph nodes, and gastrointestinal tract. Systemic mastocytosis is commonly seen in adults. These diseases are categorized on the basis of clinical features, pathologic findings, and prognosis.
A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states.
A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver.
The GENETIC TRANSLATION products of the fusion between an ONCOGENE and another gene. The latter may be of viral or cellular origin.
Adrenal cortex hormones are steroid hormones produced by the outer portion of the adrenal gland, consisting of glucocorticoids, mineralocorticoids, and androgens, which play crucial roles in various physiological processes such as metabolism regulation, stress response, electrolyte balance, and sexual development and function.
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.
Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.
An antineoplastic agent that inhibits DNA synthesis through the inhibition of ribonucleoside diphosphate reductase.
The number of WHITE BLOOD CELLS per unit volume in venous BLOOD. A differential leukocyte count measures the relative numbers of the different types of white cells.

Clonality of isolated eosinophils in the hypereosinophilic syndrome. (1/187)

The idiopathic hypereosinophilic syndrome (IHES) is a rare disorder characterized by unexplained, persistent eosinophilia associated with multiple organ dysfunction due to eosinophilic tissue infiltration. In the absence of karyotypic abnormalities, there is no specific test to detect clonal eosinophilia in IHES. Analysis of X-chromosome inactivation patterns can be used to determine whether proliferative disorders are clonal in origin. Methylation of HpaII and Hha I sites near the polymorphic trinucleotide repeat of the human androgen receptor gene (HUMARA) has been shown to correlate with X-inactivation. In this study, we have used the polymerase chain reaction (PCR) with nested primers to analyze X-inactivation patterns of the HUMARA loci in purified eosinophils from female patients with eosinophilia. Peripheral blood eosinophils were isolated by their autofluoresence using flow cytometric sorting. Eosinophils purified from a female patient presenting with IHES were found to show a clonal pattern of X-inactivation. Eosinophil-depleted leukocytes from this patient were polyclonal by HUMARA analysis, thus excluding skewedness of random X-inactivation. After corticosteroid suppression of her blood eosinophilia, a clonal population of eosinophils could no longer be detected in purified eosinophils. In contrast, eosinophils purified from a patient with Churg-Strauss syndrome and from six patients with reactive eosinophilias attributed to allergy, parasitic infection, or drug reaction showed a polyclonal pattern of X-inactivation by HUMARA analysis. The finding of clonal eosinophilia in a patient presenting with IHES indicates that such patients may have, in reality, a low-grade clonal disorder that can be distinguished from reactive eosinophilias by HUMARA analysis. Further, the method described can be used to monitor disease progression.  (+info)

Systemic lupus erythematosus, eosinophilia and Loffler's endocarditis. An unusual association. (2/187)

A 24-yr-old male, known since the age of 11 to have a nonerosive arthritis and later diagnosed as having systemic lupus erythematosus (SLE), developed subacute heart failure with diffuse lung infiltrates and died suddenly after having presented a moderate hypereosinophilia for 6 months for which no other causes besides the SLE were found. A post mortem examination revealed Loffler's endocarditis (endocarditis parietalis fibroplastica) with acute pulmonary capillaritis. This represents Loffler's endocarditis in the setting of SLE. To the best of the authors' knowledge, this association has not been reported before.  (+info)

Allogeneic peripheral blood stem cell transplantation for hypereosinophilic syndrome with severe cardiac dysfunction. (3/187)

A 42-year-old male underwent an HLA-matched sibling PBSC transplant for hypereosinophilic syndrome (HES) diagnosed in August 1995. Prior to transplant he experienced progressive cardiac and pulmonary dysfunction with red cell and platelet transfusion dependence despite therapy with hydroxyurea, steroids and interferon. He received busulfan (16 mg/kg) and cyclophosphamide (120 mg/kg) as conditioning and standard GVHD prophylaxis with cyclosporin A and methotrexate. At day +336 he was transfusion independent without GVHD. Prompt reduction of the eosinophil count (<500/microl) and rapid improvement of cardiac function were documented, demonstrating the reversibility of organ dysfunction. Allogeneic PBSCT is an effective therapeutic option for patients with HES who fail conventional therapy.  (+info)

Hepatic involvement in hypereosinophilia: sonographic findings. (4/187)

Hypereosinophilic syndrome may cause eosinophil-related tissue damage to various organs. The purpose of this paper is to describe sonographic findings in 13 patients with hypereosinophilia in whom the liver was involved. The diagnosis in these 13 patients was based on liver biopsy in seven patients with bone marrow biopsy in six patients. Eight patients had hypereosinophilic syndrome and five patients had clonorchiasis. All 13 patients had mild to marked hepatomegaly. Seven of 13 patients showed multiple round or oval hypoechoic (n = 6) or variably echogenic (n = 1) lesions measuring 1 to 2 cm with poorly defined margins in both lobes of the liver. Four patients had one or two hypoechoic lesions 3 to 4 cm in size, with geographic pattern and poorly defined margins. Two patients showed diffuse hepatomegaly with increased parenchymal echogenicity. The number of lesions and the extent of diffuse lesions seem to be proportional to the degree of eosinophilia. Hypereosinophilia may produce multiple small focal hepatic lesions or diffuse segmental or lobar echogenic lesions simulating primary or metastatic tumor of the liver.  (+info)

AlphaIFN-induced hematologic and cytogenetic remission in chronic eosinophilic leukemia with t(1;5). (5/187)

Chronic eosinophilic leukemia (CEL) is a myeloproliferative disease characterized by excessive eosinophilic proliferation with clonal cytogenetic abnormalities. The most frequent cytogenetic abnormality is a break in the q 31-35 region of chromosome 5, where genes encoding for IL-3, IL-5 and GM-CSF (all cytokines involved in eosinophilopoiesis) are located. We report the case of a patient with CEL with t(1;5) (q23;q31), who obtained complete hematologic and major cytogenetic response after two years of alpha-interferon (alpha-IFN) therapy. Two other cases of complete response to alpha-IFN are reported in the literature. A trial with alpha-IFN could be considered as front line treatment in this rare disease.  (+info)

Abnormal clones of T cells producing interleukin-5 in idiopathic eosinophilia. (6/187)

BACKGROUND: The cause of persistent eosinophilia and the hypereosinophilic syndrome is unknown. Recent work suggests that in some patients with the hypereosinophilic syndrome, a clone of abnormal T cells produces large amounts of interleukin-5, a cytokine required for the growth and differentiation of eosinophils. We examined T-cell surface markers, rearranged T-cell-receptor genes, and in vitro production of cytokines by T cells from patients with idiopathic eosinophilia. METHODS: The expression of surface molecules on T cells was measured by flow cytometry. Cytokine expression was measured by enzyme-linked immunosorbent assay, flow cytometry, and immunohistochemical analysis. To identify dominant (clonal) rearrangements of the T-cell receptor within the lymphocyte population, Southern blot analysis (beta chain) and the polymerase chain reaction (gamma chain) were performed according to standard protocols. RESULTS: Among 60 patients with idiopathic eosinophilia, 16 had circulating T cells with an aberrant immunophenotype. In each of these patients, the abnormal immunophenotype was unique. Evidence of clonal rearrangements of the T-cell receptor was obtained in 8 of the 16 patients. In most instances, the abnormal T cells expressed large amounts of surface proteins associated with T-cell activation (the alpha chain of the interleukin-2 receptor and the HLA-DR antigen). Moreover, the aberrant T cells produced large amounts of interleukin-5 in vitro. CONCLUSIONS: Clonal populations of abnormal T cells producing interleukin-5 occur in some patients with idiopathic eosinophilia.  (+info)

Reversible cerebellar involvement in the idiopathic hypereosinophilic syndrome. (7/187)

We report a patient with cerebellar manifestations due to the idiopathic hypereosinophilic syndrome, in whom magnetic resonance imaging (MRI) showed hyper-intense lesions in both cerebellar hemispheres. Following steroids and hydroxyurea administration, the lesions on MRI disappeared, suggesting that the pathogenetic mechanism was reversible and did not cause significant structural damage. To our knowledge, the resolution of the abnormal MRI findings have not been reported to date in the idiopathic hypereosinophilic syndrome.  (+info)

Allogeneic peripheral blood cell transplantation for hypereosinophilic syndrome with myelofibrosis. (8/187)

Patients with hypereosinophilic syndrome (HES) display a very heterogeneous clinical picture ranging from asymptomatic cases to very aggressive forms. We report a 38-year-old woman with progressive HES who developed severe myelofibrosis and was treated by allogeneic stem cell transplantation, using peripheral blood (PBSCT) instead of bone marrow as the source of progenitor cells, after conditioning with cytoxan and busulphan. To the best of our knowledge, this is the first case of HES with myelofibrosis treated with PBSCT. The patient remains alive 8 months post-PBSCT, and bone marrow fibrosis has significantly decreased following transplantation. Bone Marrow Transplantation (2000) 25, 217-218.  (+info)

Hypereosinophilic Syndrome (HES) is a group of disorders characterized by persistent eosinophilia (an abnormal increase in the number of eosinophils, a type of white blood cell) leading to organ damage. The eosinophil count in the peripheral blood is typically greater than 1500 cells/μL. HES can affect various organs, including the heart, skin, nervous system, and digestive tract, causing symptoms such as shortness of breath, cough, fatigue, skin rashes, muscle weakness, and abdominal pain. The exact cause of HES is not fully understood, but it is thought to be related to abnormal production or activation of eosinophils. Treatment may include corticosteroids, immunosuppressive drugs, and targeted therapies that reduce eosinophil levels.

Eosinophilia is a medical condition characterized by an abnormally high concentration of eosinophils in the circulating blood. Eosinophils are a type of white blood cell that play an important role in the immune system, particularly in fighting off parasitic infections and regulating allergic reactions. However, when their numbers become excessively high, they can contribute to tissue damage and inflammation.

Eosinophilia is typically defined as a count of more than 500 eosinophils per microliter of blood. Mild eosinophilia (up to 1,500 cells/μL) may not cause any symptoms and may be discovered during routine blood tests. However, higher levels of eosinophilia can lead to various symptoms such as coughing, wheezing, skin rashes, and organ damage, depending on the underlying cause.

The causes of eosinophilia are varied and can include allergic reactions, parasitic infections, autoimmune disorders, certain medications, and some types of cancer. Accurate diagnosis and treatment of eosinophilia require identification and management of the underlying cause.

Eosinophils are a type of white blood cell that play an important role in the body's immune response. They are produced in the bone marrow and released into the bloodstream, where they can travel to different tissues and organs throughout the body. Eosinophils are characterized by their granules, which contain various proteins and enzymes that are toxic to parasites and can contribute to inflammation.

Eosinophils are typically associated with allergic reactions, asthma, and other inflammatory conditions. They can also be involved in the body's response to certain infections, particularly those caused by parasites such as worms. In some cases, elevated levels of eosinophils in the blood or tissues (a condition called eosinophilia) can indicate an underlying medical condition, such as a parasitic infection, autoimmune disorder, or cancer.

Eosinophils are named for their staining properties - they readily take up eosin dye, which is why they appear pink or red under the microscope. They make up only about 1-6% of circulating white blood cells in healthy individuals, but their numbers can increase significantly in response to certain triggers.

The platelet-derived growth factor receptor alpha (PDGFR-α) is a type of cell surface receptor that binds to specific proteins called platelet-derived growth factors (PDGFs). PDGFR-α is a transmembrane tyrosine kinase receptor, which means it has an intracellular portion containing tyrosine kinase enzymatic activity.

When PDGFs bind to PDGFR-α, they induce receptor dimerization and activation of the tyrosine kinase domain, leading to autophosphorylation of specific tyrosine residues on the receptor. This triggers a signaling cascade that promotes cell growth, proliferation, survival, and migration. PDGFR-α is primarily expressed in cells of mesenchymal origin, such as fibroblasts, smooth muscle cells, and glial cells.

PDGFR-α plays crucial roles during embryonic development, wound healing, and tissue repair. However, aberrant activation or mutations in PDGFR-α have been implicated in various pathological conditions, including cancer, atherosclerosis, and fibrotic disorders. Therefore, PDGFR-α is an important target for therapeutic interventions in these diseases.

mRNA cleavage and polyadenylation factors are a group of proteins that play a crucial role in the post-transcriptional modification of messenger RNA (mRNA). This process involves two main steps: mRNA cleavage and polyadenylation.

1. Cleavage: During this step, the mRNA molecule is cut at a specific site, resulting in the formation of two separate fragments. The fragment that will become the mature mRNA is called the 3' untranslated region (3' UTR).

2. Polyadenylation: Following cleavage, a string of adenine nucleotides (poly(A) tail) is added to the 3' end of the newly formed 3' UTR. This poly(A) tail plays an essential role in mRNA stability, transport from the nucleus to the cytoplasm, and translation initiation.

mRNA cleavage and polyadenylation factors include various proteins that orchestrate these events, such as:

* Cleavage and polyadenylation specificity factor (CPSF) complex: This complex recognizes and binds to the polyadenylation signal sequence in the pre-mRNA. It contains several subunits, including CPSF1, CPSF2, CPSF3, CPSF4, and CPSF7.
* Cleavage stimulation factor (CstF) complex: This complex recognizes and binds to the GU-rich region downstream of the polyadenylation signal sequence. It contains several subunits, including CstF50, CstF64, CstF77, and CstF80.
* Cleavage factors I (CFIm) and II (CFIIm): These complexes help position the CPSF complex at the correct site for cleavage and polyadenylation. CFIm contains the subunits CFIm25, CFIm59, and CFIm68, while CFIIm consists of the subunits CLIP1 and PAP73.
* Poly(A) polymerase (PAP): This enzyme adds the string of adenine residues to the 3' end of the pre-mRNA after cleavage.

Together, these factors work together to ensure accurate and efficient cleavage and polyadenylation of pre-mRNAs during gene expression.

A syndrome, in medical terms, is a set of symptoms that collectively indicate or characterize a disease, disorder, or underlying pathological process. It's essentially a collection of signs and/or symptoms that frequently occur together and can suggest a particular cause or condition, even though the exact physiological mechanisms might not be fully understood.

For example, Down syndrome is characterized by specific physical features, cognitive delays, and other developmental issues resulting from an extra copy of chromosome 21. Similarly, metabolic syndromes like diabetes mellitus type 2 involve a group of risk factors such as obesity, high blood pressure, high blood sugar, and abnormal cholesterol or triglyceride levels that collectively increase the risk of heart disease, stroke, and diabetes.

It's important to note that a syndrome is not a specific diagnosis; rather, it's a pattern of symptoms that can help guide further diagnostic evaluation and management.

Interleukin-5 (IL-5) is a type of cytokine, which is a small signaling protein that mediates and regulates immunity, inflammation, and hematopoiesis. IL-5 is primarily produced by activated T cells, especially Th2 cells, as well as mast cells, eosinophils, and innate lymphoid cells (ILCs).

The primary function of IL-5 is to regulate the growth, differentiation, activation, and survival of eosinophils, a type of white blood cell that plays a crucial role in the immune response against parasitic infections. IL-5 also enhances the ability of eosinophils to migrate from the bone marrow into the bloodstream and then into tissues, where they can participate in immune responses.

In addition to its effects on eosinophils, IL-5 has been shown to have a role in the regulation of B cell function, including promoting the survival and differentiation of B cells into antibody-secreting plasma cells. Dysregulation of IL-5 production and activity has been implicated in several diseases, including asthma, allergies, and certain parasitic infections.

Eosinophil granule proteins are a group of biologically active molecules that are stored within the granules of eosinophils, which are types of white blood cells. These proteins include:

1. Eosinophil cationic protein (ECP): A protein with potent ribonuclease activity and the ability to disrupt cell membranes. It is involved in the immune response against parasites and has been implicated in the pathogenesis of several inflammatory diseases, such as asthma and allergies.
2. Eosinophil peroxidase (EPO): An enzyme that generates hypohalous acids, which can cause oxidative damage to cells and tissues. It contributes to the microbicidal activity of eosinophils and has been implicated in the pathogenesis of various inflammatory diseases.
3. Major basic protein (MBP): A highly cationic protein that can disrupt cell membranes, leading to cell lysis. MBP is involved in the immune response against parasites and has been linked to tissue damage in several inflammatory conditions, such as asthma, chronic rhinosinusitis, and eosinophilic esophagitis.
4. Eosinophil-derived neurotoxin (EDN): A protein with ribonuclease activity that can induce histamine release from mast cells and contribute to the inflammatory response. EDN is also involved in the immune response against parasites and has been implicated in the pathogenesis of asthma, allergies, and other inflammatory diseases.

These eosinophil granule proteins are released during eosinophil activation and degranulation, which can occur in response to various stimuli, such as immune complexes, cytokines, and infectious agents. Their release contributes to the inflammatory response and can lead to tissue damage in various diseases.

Churg-Strauss syndrome (CSS), also known as eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune disorder characterized by inflammation of small- to medium-sized blood vessels (vasculitis) and the presence of eosinophils, a type of white blood cell. The syndrome typically affects multiple organ systems, including the respiratory tract, peripheral nerves, skin, heart, and kidneys.

The classic triad of symptoms includes asthma, allergies, and peripheral blood eosinophilia (high levels of eosinophils in the blood). Other common features include sinusitis, rhinitis, cough, shortness of breath, skin rashes, neuropathy (nerve damage), and cardiac involvement.

The exact cause of Churg-Strauss syndrome is not well understood, but it is believed to involve an abnormal immune response in genetically susceptible individuals. Treatment typically involves the use of immunosuppressive medications to control inflammation and prevent organ damage. Corticosteroids are often used as a first-line therapy, while other agents such as cyclophosphamide or rituximab may be added for more severe cases.

Benzamides are a class of organic compounds that consist of a benzene ring (a aromatic hydrocarbon) attached to an amide functional group. The amide group can be bound to various substituents, leading to a variety of benzamide derivatives with different biological activities.

In a medical context, some benzamides have been developed as drugs for the treatment of various conditions. For example, danzol (a benzamide derivative) is used as a hormonal therapy for endometriosis and breast cancer. Additionally, other benzamides such as sulpiride and amisulpride are used as antipsychotic medications for the treatment of schizophrenia and related disorders.

It's important to note that while some benzamides have therapeutic uses, others may be toxic or have adverse effects, so they should only be used under the supervision of a medical professional.

Chemokine (C-C motif) ligand 17 (CCL17), also known as thymus and activation-regulated chemokine (TARC), is a small signaling protein that belongs to the CC chemokine family. Chemokines are a group of cytokines, or cell signaling molecules, that play an important role in immune function by recruiting immune cells to sites of infection or inflammation.

CCL17 is produced by various types of cells, including dendritic cells, macrophages, and endothelial cells, in response to stimulation by pro-inflammatory cytokines such as interleukin (IL)-4 and IL-13. CCL17 binds to its receptor, CCR4, which is expressed on the surface of Th2 cells, regulatory T cells, and some other immune cells.

CCL17 plays a role in the recruitment of these cells to sites of inflammation, and has been implicated in the pathogenesis of various diseases, including allergies, asthma, atopic dermatitis, and certain types of cancer. In particular, CCL17 has been shown to promote the migration of Th2 cells, which are involved in the immune response to parasites and allergens, to sites of inflammation.

In addition to its role in immune function, CCL17 has also been found to have angiogenic properties, meaning it can stimulate the growth of new blood vessels. This has led to interest in its potential as a therapeutic target for diseases characterized by abnormal blood vessel formation, such as cancer and diabetic retinopathy.

Endomyocardial fibrosis is a rare heart condition characterized by the thickening and scarring (fibrosis) of the inner layer of the heart muscle (endocardium) and the muscular walls of the lower chambers of the heart (ventricles). This process can restrict the heart's ability to fill properly with blood, leading to symptoms such as shortness of breath, fatigue, and fluid retention. The exact cause of endomyocardial fibrosis is not fully understood, but it is believed to involve an abnormal immune response or inflammation. It is more commonly found in tropical regions of Africa and Asia. Treatment typically involves medications to manage symptoms and improve heart function, as well as potentially surgical interventions to remove the scar tissue and restore normal heart function.

Piperazines are a class of heterocyclic organic compounds that contain a seven-membered ring with two nitrogen atoms at positions 1 and 4. They have the molecular formula N-NRR' where R and R' can be alkyl or aryl groups. Piperazines have a wide range of uses in pharmaceuticals, agrochemicals, and as building blocks in organic synthesis.

In a medical context, piperazines are used in the manufacture of various drugs, including some antipsychotics, antidepressants, antihistamines, and anti-worm medications. For example, the antipsychotic drug trifluoperazine and the antidepressant drug nefazodone both contain a piperazine ring in their chemical structure.

However, it's important to note that some piperazines are also used as recreational drugs due to their stimulant and euphoric effects. These include compounds such as BZP (benzylpiperazine) and TFMPP (trifluoromethylphenylpiperazine), which have been linked to serious health risks, including addiction, seizures, and death. Therefore, the use of these substances should be avoided.

Pyrimidines are heterocyclic aromatic organic compounds similar to benzene and pyridine, containing two nitrogen atoms at positions 1 and 3 of the six-member ring. They are one of the two types of nucleobases found in nucleic acids, the other being purines. The pyrimidine bases include cytosine (C) and thymine (T) in DNA, and uracil (U) in RNA, which pair with guanine (G) and adenine (A), respectively, through hydrogen bonding to form the double helix structure of nucleic acids. Pyrimidines are also found in many other biomolecules and have various roles in cellular metabolism and genetic regulation.

Mastocytosis is a group of rare disorders caused by the accumulation of abnormal number of mast cells in various tissues of the body, particularly the skin and internal organs such as the bone marrow, liver, spleen, and gastrointestinal tract. Mast cells are types of white blood cells that play an important role in the immune system, releasing chemicals like histamine, heparin, and leukotrienes during allergic reactions or injury to help protect the body. However, excessive accumulation of mast cells can lead to chronic inflammation, tissue damage, and various symptoms.

There are two main types of mastocytosis: cutaneous mastocytosis (CM) and systemic mastocytosis (SM). CM primarily affects the skin, causing redness, itching, hives, and other skin abnormalities. SM, on the other hand, involves internal organs and can be more severe, with symptoms such as diarrhea, stomach pain, fatigue, bone pain, and anaphylaxis (a life-threatening allergic reaction).

Mastocytosis is typically caused by genetic mutations that lead to the overproduction of mast cells. The diagnosis of mastocytosis usually involves a combination of physical examination, medical history, blood tests, skin biopsy, and bone marrow aspiration. Treatment options depend on the type and severity of the disease and may include antihistamines, corticosteroids, chemotherapy, targeted therapy, and in severe cases, stem cell transplantation.

Pulmonary eosinophilia is a condition characterized by an increased number of eosinophils, a type of white blood cell, in the lungs or pulmonary tissues. Eosinophils play a role in the body's immune response to parasites and allergens, but an overabundance can contribute to inflammation and damage in the lungs.

The condition may be associated with various underlying causes, such as:

1. Asthma or allergic bronchopulmonary aspergillosis (ABPA)
2. Eosinophilic lung diseases, like eosinophilic pneumonia or idiopathic hypereosinophilic syndrome
3. Parasitic infections, such as ascariasis or strongyloidiasis
4. Drug reactions, including certain antibiotics and anti-inflammatory drugs
5. Connective tissue disorders, like rheumatoid arthritis or Churg-Strauss syndrome
6. Malignancies, such as lymphoma or leukemia
7. Other less common conditions, like tropical pulmonary eosinophilia or cryptogenic organizing pneumonia

Symptoms of pulmonary eosinophilia can vary but often include cough, shortness of breath, wheezing, and chest discomfort. Diagnosis typically involves a combination of clinical evaluation, imaging studies, and laboratory tests, such as complete blood count (CBC) with differential, bronchoalveolar lavage (BAL), or lung biopsy. Treatment depends on the underlying cause and may include corticosteroids, antibiotics, or antiparasitic medications.

Systemic mastocytosis is a rare group of diseases characterized by the accumulation of abnormal number of mast cells in various organs and tissues of the body. Mast cells are a type of white blood cell that plays an important role in the immune system, particularly in allergic reactions and inflammation. In systemic mastocytosis, the excessive buildup of mast cells can cause a range of symptoms such as skin rashes, itching, gastrointestinal disturbances, bone pain, and in severe cases, organ damage or failure.

The diagnosis of systemic mastocytosis typically involves a combination of clinical evaluation, laboratory tests, imaging studies, and sometimes biopsies to confirm the presence of abnormal mast cells. Treatment for systemic mastocytosis depends on the severity and extent of the disease, but may include medications to manage symptoms, reduce mast cell activation and proliferation, and prevent complications. In some cases, cytoreductive therapies such as chemotherapy or stem cell transplantation may be recommended.

Prednisolone is a synthetic glucocorticoid drug, which is a class of steroid hormones. It is commonly used in the treatment of various inflammatory and autoimmune conditions due to its potent anti-inflammatory and immunosuppressive effects. Prednisolone works by binding to specific receptors in cells, leading to changes in gene expression that reduce the production of substances involved in inflammation, such as cytokines and prostaglandins.

Prednisolone is available in various forms, including tablets, syrups, and injectable solutions. It can be used to treat a wide range of medical conditions, including asthma, rheumatoid arthritis, inflammatory bowel disease, allergies, skin conditions, and certain types of cancer.

Like other steroid medications, prednisolone can have significant side effects if used in high doses or for long periods of time. These may include weight gain, mood changes, increased risk of infections, osteoporosis, diabetes, and adrenal suppression. As a result, the use of prednisolone should be closely monitored by a healthcare professional to ensure that its benefits outweigh its risks.

Prednisone is a synthetic glucocorticoid, which is a type of corticosteroid hormone. It is primarily used to reduce inflammation in various conditions such as asthma, allergies, arthritis, and autoimmune disorders. Prednisone works by mimicking the effects of natural hormones produced by the adrenal glands, suppressing the immune system's response and reducing the release of substances that cause inflammation.

It is available in oral tablet form and is typically prescribed to be taken at specific times during the day, depending on the condition being treated. Common side effects of prednisone include increased appetite, weight gain, mood changes, insomnia, and easy bruising. Long-term use or high doses can lead to more serious side effects such as osteoporosis, diabetes, cataracts, and increased susceptibility to infections.

Healthcare providers closely monitor patients taking prednisone for extended periods to minimize the risk of adverse effects. It is essential to follow the prescribed dosage regimen and not discontinue the medication abruptly without medical supervision, as this can lead to withdrawal symptoms or a rebound of the underlying condition.

An oncogene protein fusion is a result of a genetic alteration in which parts of two different genes combine to create a hybrid gene that can contribute to the development of cancer. This fusion can lead to the production of an abnormal protein that promotes uncontrolled cell growth and division, ultimately resulting in a malignant tumor. Oncogene protein fusions are often caused by chromosomal rearrangements such as translocations, inversions, or deletions and are commonly found in various types of cancer, including leukemia and sarcoma. These genetic alterations can serve as potential targets for cancer diagnosis and therapy.

The adrenal cortex hormones are a group of steroid hormones produced and released by the outer portion (cortex) of the adrenal glands, which are located on top of each kidney. These hormones play crucial roles in regulating various physiological processes, including:

1. Glucose metabolism: Cortisol helps control blood sugar levels by increasing glucose production in the liver and reducing its uptake in peripheral tissues.
2. Protein and fat metabolism: Cortisol promotes protein breakdown and fatty acid mobilization, providing essential building blocks for energy production during stressful situations.
3. Immune response regulation: Cortisol suppresses immune function to prevent overactivation and potential damage to the body during stress.
4. Cardiovascular function: Aldosterone regulates electrolyte balance and blood pressure by promoting sodium reabsorption and potassium excretion in the kidneys.
5. Sex hormone production: The adrenal cortex produces small amounts of sex hormones, such as androgens and estrogens, which contribute to sexual development and function.
6. Growth and development: Cortisol plays a role in normal growth and development by influencing the activity of growth-promoting hormones like insulin-like growth factor 1 (IGF-1).

The main adrenal cortex hormones include:

1. Glucocorticoids: Cortisol is the primary glucocorticoid, responsible for regulating metabolism and stress response.
2. Mineralocorticoids: Aldosterone is the primary mineralocorticoid, involved in electrolyte balance and blood pressure regulation.
3. Androgens: Dehydroepiandrosterone (DHEA) and its sulfate derivative (DHEAS) are the most abundant adrenal androgens, contributing to sexual development and function.
4. Estrogens: Small amounts of estrogens are produced by the adrenal cortex, mainly in women.

Disorders related to impaired adrenal cortex hormone production or regulation can lead to various clinical manifestations, such as Addison's disease (adrenal insufficiency), Cushing's syndrome (hypercortisolism), and congenital adrenal hyperplasia (CAH).

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.

A fatal outcome is a term used in medical context to describe a situation where a disease, injury, or illness results in the death of an individual. It is the most severe and unfortunate possible outcome of any medical condition, and is often used as a measure of the severity and prognosis of various diseases and injuries. In clinical trials and research, fatal outcome may be used as an endpoint to evaluate the effectiveness and safety of different treatments or interventions.

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.

A leukocyte count, also known as a white blood cell (WBC) count, is a laboratory test that measures the number of leukocytes in a sample of blood. Leukocytes are a vital part of the body's immune system and help fight infection and inflammation. A high or low leukocyte count may indicate an underlying medical condition, such as an infection, inflammation, or a bone marrow disorder. The normal range for a leukocyte count in adults is typically between 4,500 and 11,000 cells per microliter (mcL) of blood. However, the normal range can vary slightly depending on the laboratory and the individual's age and sex.

... on patient.info Hypereosinophilic Syndrome on eMedicine (Articles with short description, Short ... Many cases of hypereosinophilic syndrome may be treated exclusively with monoclonal antibodies such as mepolizumab It may then ... Hypereosinophilic syndrome was first described as a distinct entity by Hardy and Anderson in 1968. "OMIM Entry - # 607685 - ... Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in ...
Hypereosinophilic Syndrome (HES) Hypereosinophilic syndrome is a combination of rare complications that are explained by an ... "Hypereosinophilic syndrome , Genetic and Rare Diseases Information Center (GARD) - an NCATS Program". rarediseases.info.nih.gov ... An uncommon disorder that is known to be associated with Hypereosinophilic Syndrome is Löffler endocarditis.[citation needed] ...
Curtis C, Ogbogu P (2016). "Hypereosinophilic Syndrome". Clinical Reviews in Allergy & Immunology. 50 (2): 240-51. doi:10.1007/ ...
2003). "Discovery of a fusion kinase in EOL-1 cells and idiopathic hypereosinophilic syndrome". Proc. Natl. Acad. Sci. U.S.A. ... Cools J, Stover EH, Gilliland DG (2006). "Detection of the FIP1L1-PDGFRA Fusion in Idiopathic Hypereosinophilic Syndrome and ... Helbig G (February 2018). "Imatinib for the treatment of hypereosinophilic syndromes". Expert Review of Clinical Immunology. 14 ... Roufosse F (2015). "Management of Hypereosinophilic Syndromes". Immunology and Allergy Clinics of North America. 35 (3): 561-75 ...
Roufosse F, Cogan E, Goldman M (2004). "Recent advances in pathogenesis and management of hypereosinophilic syndromes". Allergy ... Roufosse F (2015). "Management of Hypereosinophilic Syndromes". Immunology and Allergy Clinics of North America. 35 (3): 561-75 ... Clonal analysis in an idiopathic hypereosinophilic syndrome". Journal of Immunology. 139 (11): 3753-8. doi:10.4049/jimmunol. ... "The lymphoid variant of hypereosinophilic syndrome: study of 21 patients with CD3-CD4+ aberrant T-cell phenotype". Medicine. 93 ...
The idiopathic hypereosinophilic syndrome. Secondary conditions that may lead to eosinophilic myocarditis are: Infections ... Zhang Y, Ma L (2017). "Repeated Prosthetic Mitral Valve Thrombosis in Fluke Infection Induced Hypereosinophilic Syndrome". The ... Roufosse F (2015). "Management of Hypereosinophilic Syndromes". Immunology and Allergy Clinics of North America. 35 (3): 561-75 ... of individuals diagnosed with the hypereosinophilic syndrome. Loeffler's and the latter cases had pathological features of ...
Roufosse F (2015). "Management of Hypereosinophilic Syndromes". Immunology and Allergy Clinics of North America. 35 (3): 561-75 ... Kleinfeldt, Tilo; Ince, Hueseyin; Nienaber, Christoph A. (2011). "Hypereosinophilic Syndrome: A rare case of Loeffler's ... chronic eosinophilic leukemia and the hypereosinophilic syndrome. Secondary causes (i.e. disorders in which other diseases ... This stage is dominated by signs and symptoms of the acute coronary syndrome such as angina, heart attack, and congestive heart ...
Current differential diagnosis of hypereosinophilic syndrome]". Medicinski Pregled. 60 (11-12): 581-6. doi:10.2298/MPNS0712581D ... Stevens-Johnson syndrome and erythema multiforme has been reported from use of tetrazepam. Cross-reactivity with other ... Bachmeyer C, Assier H, Roujeau JC, Blum L (July 2008). "Probable drug rash with eosinophilia and systemic symptoms syndrome ... 2 March 1998). "Stevens-Johnson syndrome from tetrazepam". Allergologia et Immunopathologia. 26 (2): 55-7. PMID 9645262. ...
Wayback Machine Hypereosinophilic Syndrome on patient.info Hypereosinophilic Syndrome on eMedicine Hypereosinophilic Syndrome ( ... Congenital disorders Hyperimmunoglobulin E syndrome Omenn syndrome Familial eosinophilia Eosinophilia-myalgia syndrome ... The idiopathic hypereosinophilic syndrome is a disorder characterized by hypereosiophilia that is associated with eosinophil- ... Roufosse F, Cogan E, Goldman M (2004). "Recent advances in pathogenesis and management of hypereosinophilic syndromes". Allergy ...
August 2, 2018). "Dexpramipexole as an oral steroid-sparing agent in hypereosinophilic syndromes". Blood. 2018 Aug 2, 132(5) (5 ... in Subjects With Hypereosinophilic Syndrome Clinicaltrials.gov:Study of Dexpramipexole Chronic Sinusitis With Nasal Polyps and ... significantly reduced eosinophil counts and glucocorticoid requirements in patients with hypereosinophilic syndrome (HES) and ...
... idiopathic hypereosinophilic syndrome (HES) if there was evidence of eosinophil-induced tissue damage but no criteria ... associated with the hypereosinophilic syndrome. The World Health Organization in 2015 included in their classification of ... the hypereosinophilic syndrome, chronic eosinophilic leukemia, or acute eosinophilic leukemia; b) myeloproliferative neoplasm/ ... "IgG4-related disease and lymphocyte-variant hypereosinophilic syndrome: A comparative case series". European Journal of ...
Fusion of PDGFRA has been found to be responsible for hematological malignances like hypereosinophilic syndrome. The ... Mutations in FLT3 have also been reported in acute lymphoblastic leukemia (ALL) and myelodysplastic syndrome (MDS). Activating ... "Internal tandem duplication of the FLT3 gene is preferentially seen in acute myeloid leukemia and myelodysplastic syndrome ... the FLT3 gene is found in acute lymphoblastic leukaemia as well as acute myeloid leukaemia but not in myelodysplastic syndrome ...
"Clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome". New England Journal of Medicine. ...
... hypereosinophilic syndrome, leukemia, myelodysplastic syndromes, other white cell dyscrasias, and gammopathies. ... Other examples of systemic disease capable of causing mouth ulcers include lupus erythematosus, Sweet syndrome, reactive ... Dermatological causes include chronic ulcerative stomatitis, erythema multiforme (Stevens-Johnson syndrome), angina bullosa ... including Behçet's syndrome". Clinical and Experimental Immunology. 156 (1): 1-11. doi:10.1111/j.1365-2249.2008.03857.x. PMC ...
It can arise de novo or may develop in patients having the chronic form of a hypereosinophilic syndrome. Patients with acute ... eosinophilic leukemia have a propensity for developing bronchospasm as well as symptoms of the acute coronary syndrome and/or ...
... with SCF to induce systemic mastocytosis in a murine model of chronic eosinophilic leukemia/hypereosinophilic syndrome". Blood ... Nimer SD (May 2008). "Myelodysplastic syndromes". Blood. 111 (10): 4841-4851. doi:10.1182/blood-2007-08-078139. PMID 18467609. ...
... hypereosinophilic syndrome (HES), chronic eosinophilic leukemia (CEL), systemic mastocytosis, and myelodysplastic syndrome. ... Imatinib has been reported to be an effective treatment for FIP1L1-PDGFRalpha+ mast cell disease, hypereosinophilic syndrome, ... hypereosinophilic syndrome and/or chronic eosinophilic leukemia who have the FIP1L1-PDGFRα fusion kinase (CHIC2 allele deletion ...
... hypereosinophilic syndrome may be considered. In cases of underlying allergic reactions or adverse sensitivity, skin rashes may ... If splenomegaly is detected, a myeloproliferative syndrome may be suspected. Intrinsically related symptoms such as fever, ...
... or hypereosinophilic syndromes.[citation needed] Minor tricuspid insufficiency is common in healthy individuals. In more severe ... and hypereosinophilic syndrome (Loeffler endocarditis). Endocarditis of the valves can lead to regurgitation through that valve ... Noonan syndrome, and congenital rubella syndrome. Unless the degree of stenosis is severe, individuals with pulmonary stenosis ... Marfan's Syndrome is a connective tissue disorder that can lead to chronic aortic or mitral regurgitation. Osteogenesis ...
... the diagnosis of idiopathic hypereosinophilic syndrome should be considered. Corticosteroids are the mainstay of therapy with a ...
... and extracellular matrix of the skin in the hypereosinophilic syndrome". Lab. Invest. 62 (5): 590-607. PMID 2160562. Dvorak AM ...
... is not a form of the idiopathic hypereosinophilic syndrome in that there is little or no evidence that it ... Gleich syndrome has a good prognosis. Attack severity may improve with steroid treatment. Gleich GJ, Schroeter AL, Marcoux JP, ... Gleich's syndrome is a rare disease in which the body swells up episodically (angioedema), associated with raised antibodies of ... It is suggested that most forms of Gleich's syndrome are due to a similar aberrant T cell mechanism and are a subtype of ...
To properly diagnose EoE, various diseases such as GERD, esophageal cancer, achalasia, hypereosinophilic syndrome, infection, ... achalasia hypereosinophilic syndrome, Crohn's disease, infections, pill esophagitis, or graft vs host disease. Endoscopy is ... Mast cell disorders such as Mast Cell Activation Syndrome or Mastocytosis are also frequently associated with it.[citation ...
... and hypereosinophilic syndrome (HES). It recognizes and blocks interleukin-5 (IL-5), a signalling protein of the immune system ... FDA expanded mepolizumab's indication to treat adults and children aged twelve years and older with hypereosinophilic syndrome ...
... and systemic symptoms Mastocytosis Lymphoproliferative hypereosinophilic syndrome Myeloproliferative hypereosinophilic syndrome ... Chronic eosinophilic pneumonia was first described by Carrington in 1969, and it is also known as Carrington syndrome. Prior to ... When eosinophilic pneumonia is caused by helminths, it is often called "Löffler's syndrome". The final group of parasites cause ... reaction Granulomatosis with polyangiitis Allergic bronchopulmonary aspergillosis Churg-Strauss syndrome Loeffler's syndrome ...
... hypereosinophilic syndrome M9970/1 Lymphoproliferative disease/disorder, NOS Post-transplant lymphoproliferative disorder, ... M9895/3 Acute myeloid leukemia multilineage dysplasia AML with/without prior myelodysplastic syndrome M9896/3 AML with t(8;21)( ... M9920/3 Acute myeloid leukemia and myelodysplastic syndrome, therapy related, NOS Therapy-related acute myeloid leukemia, ... chromosome abnormality M9989/3 Myelodysplastic syndrome, NOS Medical classification International Classification of Diseases ...
Werner's syndrome Endomyocardial Carcinoid heart disease Endomyocardial fibrosis Idiopathic Hypereosinophilic syndrome Chronic ... Hurler syndrome) Mucopolysaccharidosis type II (Hunter syndrome) Niemann-Pick disease Non-infiltrative Idiopathic Diabetic ...
... hypereosinophilic syndrome, or chronic myeloid leukemia (CML), all basophils from patients with CEL or CML, and all bone marrow ... homology screening of ESTs from a cDNA library generated from a patient diagnosed with idiopathic hypereosinophilic syndrome ...
... an early 1980s American video game publisher Hydroxyethyl starch Hypereosinophilic syndrome Hypertext Editing System Isis, an ...
... fibrosis Hypereosinophilic syndrome Endocrine Diabetes mellitus Hyperthyroidism Acromegaly Cardiofacial Noonan syndrome ... Left ventricular noncompaction Ion Channelopathies like the Long QT syndrome and the very rare Short QT syndrome ... Broken heart syndrome is caused by extreme emotional or physical stress. Treatment depends on the type of cardiomyopathy and ... Lipshultz, Steven E.; Messiah, Sarah E.; Miller, Tracie L. (5 April 2012). Pediatric Metabolic Syndrome: Comprehensive Clinical ...
Hypereosinophilic Syndrome on patient.info Hypereosinophilic Syndrome on eMedicine (Articles with short description, Short ... Many cases of hypereosinophilic syndrome may be treated exclusively with monoclonal antibodies such as mepolizumab It may then ... Hypereosinophilic syndrome was first described as a distinct entity by Hardy and Anderson in 1968. "OMIM Entry - # 607685 - ... Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in ...
Hypereosinophilic syndrome varies from an asymptomatic phenomenon to a life-threatening multisystem disease. It is ... encoded search term (Pediatric Hypereosinophilic Syndrome) and Pediatric Hypereosinophilic Syndrome What to Read Next on ... Hypereosinophilic syndrome has a 55.3% male predominance in the pediatric population. [6] The male-to-female ratio is 9:1 in ... Pediatric Hypereosinophilic Syndrome. Updated: Aug 02, 2018 * Author: Bruce M Rothschild, MD; Chief Editor: Harumi Jyonouchi, ...
... Editorial Article Overview authors * Boulware, D. R. ...
MPN-024 Hypereosinophilic Syndrome: A Catastrophic Pediatric Case published in the journal Clin Lymphoma Myeloma Leuk. 2022 ... MPN-024 Hypereosinophilic Syndrome: A Catastrophic Pediatric Case MPN-024 Hypereosinophilic Syndrome: A Catastrophic Pediatric ...
FDA Approves Nucala as the First and Only Biologic Treatment for Hypereosinophilic Syndrome (HES) * Third US indication for ... About Hypereosinophilic Syndrome (HES) HES is a rare and under-diagnosed disorder, making it difficult to estimate its overall ... In a clinical trial in patients with Hypereosinophilic Syndrome, no additional adverse reactions were identified to those ... for the treatment of adult and pediatric patients aged 12 years and older with Hypereosinophilic Syndrome (HES) for ≥ six ...
Fletcher S, Bain B. Diagnosis and treatment of hypereosinophilic syndromes. Curr Opin Hematol. 2007;14:37-42. [PubMed] [DOI] [ ... idiopathic hypereosinophilic syndrome (HES), lymphoproliferative disorder (T/natural killer cells), paraneoplastic syndrome. ... The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975;54:1-27. [ ... Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib Paolo Fraticelli, ...
Material/Methods: A rare case of hypereosinophilic syndrome (HES) with organ involvement in a 2-year-old child is presented. ... the definition of hypereosinophilic syndrome and eosinophilic leukemia are introduced. ... Hypereosinophilic syndrome in a 2-year old boy - a case report Med Sci Monit 2003; 9(4): 99-102 :: ID: 428662 ...
Idiopathic hypereosinophilic syndrome with cutaneous involvement: a comparative review of 32 cases ... Idiopathic hypereosinophilic syndrome with cutaneous involvement: a comparative review of 32 cases ... Idiopathic hypereosinophilic syndrome with cutaneous involvement: a comparative review of 32 cases ...
Although idiopathic hypereosinophilic syndrome (HES) is uncommon, we studied the clinical characteristics of this disorder in ... Idiopathic hypereosinophilic syndrome with cutaneous involvement: a comparative review of 32 cases ... Idiopathic hypereosinophilic syndrome with cutaneous involvement: a comparative review of 32 cases ...
Impressive combination of multiple sinus valsalvae and coronary aneurysms due to hypereosinophilic syndrome ... Impressive combination of multiple sinus valsalvae and coronary aneurysms due to hypereosinophilic syndrome. Annals of Thoracic ...
... is well-documented in patients with hypereosinophilic syndromes (HES) and detectable rearrangements of platelet-derived growth ...
... weeks has been recently approved as an add-on therapy for patients with uncontrolled hypereosinophilic syndrome (HES) without ... Hypereosinophilic syndrome (HES) is a rare condition defined by persistent blood eosinophil count , 1.5 × 109/L and evidence of ... Caminati, M., Maule, M., Benoni, R. et al. Low-dose anti-IL 5 treatment in idiopathic hypereosinophilic syndrome: towards a ... Mepolizumab reduces hypereosinophilic syndrome flares irrespective of blood eosinophil count and interleukin-5. J Allergy Clin ...
... but Hardy and Anderson first described the specific syndrome in 1968. ... Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is ... Hypereosinophilic syndrome is rare in children. The incidence of hypereosinophilic syndrome seems to decrease in the elderly ... encoded search term (Hypereosinophilic Syndrome) and Hypereosinophilic Syndrome What to Read Next on Medscape ...
Hypereosinophilic Syndrome Market is expected to grow at a high CAGR during the forecast period 2023-2030 , DataM Intelligence ... Hypereosinophilic-syndrome-market. Hypereosinophilic Syndrome Market Size, Share, Industry, Forecast and outlook (2023-2030). ...
Hypereosinophilic Syndrome - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the MSD Manuals - Medical ... Clonal hypereosinophilic syndromes There are two broad subtypes of clonal hypereosinophilic syndrome (see table Clonal ... Idiopathic hypereosinophilic syndrome Idiopathic hypereosinophilic syndrome is rare, has an unknown prevalence, and most often ... see table Abnormalities in Patients With Hypereosinophilic Syndrome Abnormalities in Patients With Hypereosinophilic Syndrome ...
"Reversible dementia with idiopathic hypereosinophilic syndrome",. abstract = "A 66-year-old woman with hypereosinophilic ... Kaplan PW, Waterbury L, Kawas C, Bolla-Wilson K, Durack D. Reversible dementia with idiopathic hypereosinophilic syndrome. ... Reversible dementia with idiopathic hypereosinophilic syndrome. P. W. Kaplan, L. Waterbury, C. Kawas, K. Bolla-Wilson, D. ... Reversible dementia with idiopathic hypereosinophilic syndrome. / Kaplan, P. W.; Waterbury, L.; Kawas, C. et al. In: Neurology ...
Hypereosinophilic syndrome: Cause of prosthetic valve obstruction. / Arsiwala, Saify; Peek, Giles; Davies, Mike et al. In: The ... Hypereosinophilic syndrome : Cause of prosthetic valve obstruction. In: The Journal of Thoracic and Cardiovascular Surgery. ... Arsiwala S, Peek G, Davies M, Sosnoski A, Firmin R. Hypereosinophilic syndrome: Cause of prosthetic valve obstruction. The ... Hypereosinophilic syndrome: Cause of prosthetic valve obstruction. Saify Arsiwala, Giles Peek, Mike Davies, Andrew Sosnoski, ...
Hypereosinophilic hyper IgE syndrome - A rare case report. Author: Sneka, P., Arun Kumar, Nachammai, S. M. and Anbu N. Aravazhi ... The onset of this syndrome may occur at any time from early childhood onwards, and may include eosinophilia and osteoarticular ... Hyper immunoglobulin (Ig) E syndrome is a rare complex immunoregulatory disorder characterized by hyperglobulinemia, recurrent ...
Hypereosinophilic syndrome. Disease definition Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of ...
Since hypereosinophilic syndrome (HES) with organ damage can appear as long as 8-9 years after the presence of a ... Since hypereosinophilic syndrome (HES) with organ damage can appear as long as 8-9 years after the presence of a ... Benign idiopathic hypereosinophilia: a feeble masquerader or a smouldering form of the hypereosinophilic syndrome?. ... hypereosinophilic state, the absolutely benign nature of our patients condition still cannot be defined. Thus, there is the ...
Roufosse FE, Goldman M, Cogan E. Hypereosinophilic syndromes. Orphanet J Rare Dis. 2007 Sep 11;2:37. doi: 10.1186/1750-1172-2- ... These two conditions have very similar signs and symptoms; however, the cause of hypereosinophilic syndrome is unknown. ... PDGFRA-associated chronic eosinophilic leukemia is often grouped with a related condition called hypereosinophilic syndrome. ... Novel imatinib-sensitive PDGFRA-activating point mutations in hypereosinophilic syndrome induce growth factor independence and ...
The effect of mepolizumab on the lungs in a boy with hypereosinophilic syndrome. / Domany, Keren Armoni; Shiran, Shelly I.; ... The effect of mepolizumab on the lungs in a boy with hypereosinophilic syndrome. In: American Journal of Respiratory and ... The effect of mepolizumab on the lungs in a boy with hypereosinophilic syndrome. American Journal of Respiratory and Critical ... title = "The effect of mepolizumab on the lungs in a boy with hypereosinophilic syndrome", ...
Hypereosinophilic Syndrome. Indicated for adults with hypereosinophilic syndrome (HES) for 6 months without an identifiable ... Hypereosinophilic Syndrome. Indicated for adults and pediatric patients aged ≥12 years with hypereosinophilic syndrome (HES) ... Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome). Indicated for adults with eosinophilic granulomatosis ...
Understanding Hypereosinophilic Syndrome. Hypereosinophilic syndrome (HES) refers to a group of blood disorders caused by high ... Cytokine Release Syndrome: Symptoms, Causes, Treatments. Cytokine release syndrome is an immune system response that can occur ...
Hypereosinophilic syndromes: an update.﻽. Liesveld JL, Abboud CN. International journal of clinical & laboratory research. 1992 ... State of the art; the hypereosinophilic syndromes.﻽. Liesveld JL, Abboud CN. Blood reviews.. 1991 March 5 (1):29-37. Epub 1900 ... Improvement in a patient with hypereosinophilic syndrome after initiation of dupilumab treatment.﻽. Wieser JK, Kuehn GJ, ... Myasthenic (Eaton-Lambert) syndrome in association with an indolent non-Hodgkins lymphoma.﻽. Liesveld JL, Stern A, Rosenthal ...
Hypereosinophilic syndrome in a 2-year old boy - a case report Małgorzata Szumera, Maria Korzon, [...] Jadwiga Krzysztofowicz, ... Hypereosinophilic syndrome in a 2-year old boy - a case report Małgorzata Szumera, Maria Korzon, Jadwiga Krzysztofowicz, Anna ... Munchausen by proxy syndrome - a case of a 3.5-year-old ill-treated boy Anna Galińska, Anna Borkowska, [...] Jolanta ... Munchausen by proxy syndrome - a case of a 3.5-year-old ill-treated boy Anna Galińska, Anna Borkowska, Jolanta Mikołajewicz, ...
An FDA-approved drug dramatically improved the health of people with rare chronic immune disorders called hypereosinophilic ... Hypereosinophilic syndromes (HES) are conditions caused by higher-than-normal numbers of white blood cells, called eosinophils ... References: Benralizumab for PDGFRA-Negative Hypereosinophilic Syndrome. Kuang FL, Legrand F, Makiya M, Ware J, Wetzler L, ... "This promising treatment advance for people with hypereosinophilic syndromes is just one example of how NIH research responds ...
Hypereosinophilic syndrome: an update. Wilkins HJ, Crane MM, Copeland K, Williams WV. Wilkins HJ, et al. Among authors: crane ...
Hypereosinophilic syndrome is a rare disease presenting with idiopathic eosinophilia and multiple organ involvement, including ... The diagnosis of idiopathic hypereosinophilic syndrome poses a dilemma because clinical manifestation and serum biomarkers are ... Since glucocorticoids are a standard therapy for both idiopathic hypereosinophilic syndrome and eosinophilic granulomatosis ... Although the relationship between asymptomatic coronavirus disease 2019 and acute idiopathic hypereosinophilic syndrome ...
Volumetric and strain analysis of the right atrium in hypereosinophilic syndrome - a three-dimensional speckle-tracking ...
  • In platelet-derived growth factor receptor alpha (PDGFRA)-associated hypereosinophilic syndrome, eosinophilia is associated with formation of the FLIP1L1/PDFGRA fusion gene, with increases in tyrosine kinase (TK) activity of PDGFRA. (medscape.com)
  • The high efficacy of tyrosine kinase inhibitor, imatinib mesylate (IM), is well-documented in patients with hypereosinophilic syndromes (HES) and detectable rearrangements of platelet-derived growth factor receptor α or β (PDGFRA/B) [1]. (efim.org)
  • Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm 3 , a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. (wjgnet.com)
  • The literature now favors the view that cases of idiopathic hypereosinophilic syndrome with FIP1L1 indeed represent chronic eosinophilic leukemia, because these patients have a molecular genetic abnormality, specifically an FIP1L1-PDGFRA fusion gene. (medscape.com)
  • Hyper immunoglobulin (Ig) E syndrome is a rare complex immunoregulatory disorder characterized by hyperglobulinemia, recurrent bacterial infections and chronic eczematous dermatitis. (journalcra.com)
  • PDGFRA -associated chronic eosinophilic leukemia is often grouped with a related condition called hypereosinophilic syndrome. (medlineplus.gov)
  • An FDA-approved drug dramatically improved the health of people with rare chronic immune disorders called hypereosinophilic syndromes. (nih.gov)
  • Hypereosinophilic syndrome (HES) and or chronic eosinophilic leukaemia (CEL). (mydr.com.au)
  • An interstitial deletion del(4)(q12q12) generating a FIP1L1-PDGFRA fusion gene is observed in diverse eosinophilia-associated hematologic disorders like hyperseosinophilic syndrome (HES), systemic mastocytosis (SM) and chronic eosinophilic leukemia (CEL). (atlasgeneticsoncology.org)
  • blood disorders, such as advanced hypereosinophilic syndrome (HES) and chronic eosinophilic leukaemia (CEL). (macmillan.org.uk)
  • Hypereosinophilic syndrome (HES) is a myeloproliferative disorder (MPD) characterized by persistent eosinophilia that is associated with damage to multiple organs. (medscape.com)
  • Elling C, Erben P, Walz C, Frickenhaus M, Schemionek M, Stehling M. Novel imatinib-sensitive PDGFRA-activating point mutations in hypereosinophilic syndrome induce growth factor independence and leukemia-like disease. (medscape.com)
  • Fraticelli P, Kafyeke A, Mattioli M, Martino GP, Murri M, Gabrielli A. Idiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib. (wjgnet.com)
  • Imatinib mesylate for unmutated hypereosinophilic syndromes: Does it work? (efim.org)
  • Treatment may include prednisone and sometimes imatinib but depends on the specific subtype of hypereosinophilic syndrome. (msdmanuals.com)
  • FIP1L1-PDGFRA associated hypereosinophilic disorders are sensitive to treatments with tyrosine kinase inhibitors such as imatinib mesylate (imatinib). (atlasgeneticsoncology.org)
  • The diagnosis of idiopathic hypereosinophilic syndrome poses a dilemma because clinical manifestation and serum biomarkers are similar to those of eosinophilic granulomatosis with polyangiitis. (biomedcentral.com)
  • Only a few cases have been reported where coronavirus disease 2019 may have caused the new onset or exacerbation of eosinophilic granulomatosis with polyangiitis or idiopathic hypereosinophilic syndrome. (biomedcentral.com)
  • Since glucocorticoids are a standard therapy for both idiopathic hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis, we initiated glucocorticoids following a multidisciplinary discussion. (biomedcentral.com)
  • Given the patient's history of recurrent sinusitis, asthma, eosinophilia, and coronary aneurysms, we diagnosed eosinophilic granulomatosis with polyangiitis ( Churg-Strauss syndrome , CSS) and cardiac involvement, and recommended high-dose corticosteroid treatment. (medscape.com)
  • subtypes of cutaneous T-cell lymphoma, SS is a malignant neoplasm originating the most common of which are mycosis from T lymphocytes, which involves the fungoides and Sézary syndrome (SS). (bvsalud.org)
  • abstract = "A 66-year-old woman with hypereosinophilic syndrome became rapidly demented. (johnshopkins.edu)
  • LONDON--( BUSINESS WIRE )--GlaxoSmithKline plc (GSK) (LSE/NYSE: GSK) today announced the US Food and Drug Administration (FDA) has approved Nucala (mepolizumab) for the treatment of adult and pediatric patients aged 12 years and older with Hypereosinophilic Syndrome (HES) for ≥ six months without an identifiable non-hematologic secondary cause. (businesswire.com)
  • Although idiopathic hypereosinophilic syndrome (HES) is uncommon, we studied the clinical characteristics of this disorder in patients with cutaneous involvement. (bmj.com)
  • Mepolizumab at the dose of 300 mg/4 weeks has been recently approved as an add-on therapy for patients with uncontrolled hypereosinophilic syndrome (HES) without any identifiable non-hematologic secondary cause. (biomedcentral.com)
  • However, long-term follow-up and X-linked clonality studies indicate that at least some patients with hypereosinophilic syndrome have an underlying clonal myeloid malignancy or a clonal or phenotypically abnormal T-cell population, suggesting a true secondary process. (medscape.com)
  • In addition, some patients with hypereosinophilic syndrome present with features typical of MPDs, such as hepatosplenomegaly, the presence of leukocyte precursors in the peripheral blood, increased alkaline phosphatase level, chromosomal abnormalities, and reticulin fibrosis. (medscape.com)
  • One limitation of the traditional definition is that it does not include those patients with some of the same abnormalities (eg, chromosomal defects) that are known causes of hypereosinophilic syndrome but who do not fulfill the traditional hypereosinophilic syndrome definition for degree or duration of eosinophilia. (msdmanuals.com)
  • Another limitation is that some patients with eosinophilia and organ damage that characterize hypereosinophilic syndrome require treatment earlier than the 6 months necessary to confirm the traditional diagnostic criteria. (msdmanuals.com)
  • This is a multicenter, open-label, Phase 1/2a dose escalation and expansion study of orally administered emavusertib (CA-4948) monotherapy in adult patients with Acute Myelogenous Leukemia (AML) or high risk Myelodysplastic Syndrome (MDS). (rochester.edu)
  • Patients with hypereosinophilic syndrome historically carried a poor prognosis before the successful therapeutic application of tyrosine kinase inhibitors. (atlasgeneticsoncology.org)
  • Benign idiopathic hypereosinophilia: a feeble masquerader or a smouldering form of the hypereosinophilic syndrome? (unipv.it)
  • Extreme hypereosinophilia with multiple abnormal findings including pulmonary ground-glass opacity lesions and mononeuritis multiplex was consistent with hypereosinophilic syndrome. (biomedcentral.com)
  • Eosinophilia, hypereosinophilia, and hypereosinophilic syndrome. (mayoclinic.org)
  • Normal cellularity with eosinophilic proliferation in the bone marrow and negative FIP1L1 - PDGFRA raised the diagnosis of idiopathic hypereosinophilic syndrome. (biomedcentral.com)
  • Eosinophilic colitis and hypereosinophilic syndrome with colic involvement are rare diagnosis that are characterized by wide-ranging gastrointestinal symptoms and idiopathic infiltration of eosinophils in the colon. (karger.com)
  • Eventually, a diagnosis of idiopathic hypereosinophilic syndrome was made. (cdc.gov)
  • A bone marrow smear was performed, in which 'cerebriform' cells were identified, confirming the diagnosis of Sézary syndrome. (bvsalud.org)
  • Hypereosinophilic syndrome is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow. (wikipedia.org)
  • One report documents a case of eosinophilia (WBC count, 80,000/μ L with 63% eosinophils) in an infant born to a mother with hypereosinophilic syndrome. (medscape.com)
  • Hypereosinophilic syndromes (HES) are conditions caused by higher-than-normal numbers of white blood cells, called eosinophils. (nih.gov)
  • 3.?Dialogue Hypereosinophilic symptoms (HES) was redefined this year 2010 as a lot more than 1500/mm3 eosinophils with out a discernible extra trigger (eg, HIV disease, worm or parasite infection, allergic illnesses, medication allergies, and nonhematologic malignancies). (rawveronica.com)
  • Hypereosinophilic syndrome was previously considered to be idiopathic, but molecular characterization has revealed that many cases have specific clonal disorders. (msdmanuals.com)
  • Hypereosinophilic syndrome (HES) is characterized by sustained non-clonal blood and tissue eosinophilia, leading to end-organ damage. (elsevierpure.com)
  • The cause of FIP1L1-PDGFRA associated hypereosinophilic syndrome is unknown as well as its association with predominantly male sex. (atlasgeneticsoncology.org)
  • Many cases of hypereosinophilic syndrome may be treated exclusively with monoclonal antibodies such as mepolizumab It may then be typical not to prescribe corticosteroids for this condition, reducing the burden of the side effects associated with corticosteroids. (wikipedia.org)
  • Yamak O, Kwiatkowski V , Navalpakam A., Poowuttikul P. "IL6ST Hyper IgE Syndrome: A Case Series of Three Related Family Members with Identical Mutations and Variable Presentations. (childrensdmc.org)
  • Hypereosinophilic syndrome is a rare disease presenting with idiopathic eosinophilia and multiple organ involvement, including the skin, lungs, gastrointestinal tract, heart, and nervous system. (biomedcentral.com)
  • [ 15 ] In addition, there are documented cases of acute transformation to either AML or granulocytic sarcoma in some cases of hypereosinophilic syndrome after an interval as long as 24 years. (medscape.com)
  • Although the relationship between asymptomatic coronavirus disease 2019 and acute idiopathic hypereosinophilic syndrome exacerbation was uncertain, the chronological order of the symptomatic development suggested a possible link. (biomedcentral.com)
  • She had a positive polymerase chain reaction (PCR) test for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) 5 weeks before her visit, which was detected because she was in close contact with her husband who had COVID-19. (biomedcentral.com)
  • Some possible symptoms a patient may present with include: Cardiomyopathy Skin lesions Thromboembolic disease Pulmonary disease Neuropathy Hepatosplenomegaly Reduced ventricular size Atopic eczema Numerous techniques are used to diagnose hypereosinophilic syndrome, of which the most important is blood testing. (wikipedia.org)
  • Hypereosinophilic syndrome varies from an asymptomatic phenomenon to a life-threatening multisystem disease. (medscape.com)
  • We present the case of a 48-year-old Japanese woman with history of asthma who developed deteriorating symptoms of insidiously developed idiopathic hypereosinophilic syndrome following asymptomatic coronavirus disease 2019. (biomedcentral.com)
  • Guillain-Barré syndrome and multisystem inflammatory syndrome in children (MIS-C), which are similar to Kawasaki disease, are well-known complications. (biomedcentral.com)
  • Hypereosinophilic syndrome (HES) constitutes a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia and/or tissue eosinophilia associated with a wide range of clinical manifestations reflecting eosinophil-induced tissue/organ damage. (orpha.net)
  • Since hypereosinophilic syndrome (HES) with organ damage can appear as long as 8-9 years after the presence of a hypereosinophilic state, the absolutely benign nature of our patient's condition still cannot be defined. (unipv.it)
  • Hypereosinophilic syndrome was first described as a distinct entity by Hardy and Anderson in 1968. (wikipedia.org)
  • General references Hypereosinophilic syndrome is a condition characterized by peripheral blood eosinophilia with manifestations of organ system involvement or dysfunction directly related to eosinophilia in the. (msdmanuals.com)
  • [ 2 ] Hypereosinophilic syndrome is very rare in children. (medscape.com)
  • This promising treatment advance for people with hypereosinophilic syndromes is just one example of how NIH research responds to the unique medical needs of individuals with rare diseases. (nih.gov)
  • Kwiatkowski V , Navalpakam A., Poowuttikul P. "Idiopathic Hypereosinophilic Syndrome Diagnosed in an Infant: A Case Report. (childrensdmc.org)
  • The onset of this syndrome may occur at any time from early childhood onwards, and may include eosinophilia and osteoarticular and dental abnormalities. (journalcra.com)
  • Initial management consisted of en una paciente con topical corticosteroids and oral antihistamines with little clinical response. (bvsalud.org)
  • She had a history of bronchial asthma, allergic rhinitis, and oral allergy syndrome to melon and watermelon with eosinophilia. (biomedcentral.com)
  • However, due to advances in the diagnostic techniques, secondary causes of eosinophilia can be identified in a proportion of cases that would have otherwise been classified as idiopathic hypereosinophilic syndrome. (medscape.com)
  • Persons aged 5-80 years can have hypereosinophilic syndrome. (medscape.com)
  • Respiratory failure in a patient plus respiratory distress associated with bilateral basal rales, presented respiratory failure, and during induction of mechanical with Sézary syndrome. (bvsalud.org)