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 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.
Skin lesions due to abnormal infiltration of MAST CELLS. Cutaneous mastocytosis is confined to the skin without the involvement of other tissues or organs, and is mostly found in children. The three major variants are: URTICARIA PIGMENTOSA; diffuse cutaneous mastocytosis; and SOLITARY MASTOCYTOMA OF SKIN.
The most common form of cutaneous mastocytosis (MASTOCYTOSIS, CUTANEOUS) that occurs primarily in children. It is characterized by the multiple small reddish-brown pigmented pruritic macules and papules.
A family of neutral serine proteases with TRYPSIN-like activity. Tryptases are primarily found in the SECRETORY GRANULES of MAST CELLS and are released during mast cell degranulation.
A protein-tyrosine kinase receptor that is specific for STEM CELL FACTOR. This interaction is crucial for the development of hematopoietic, gonadal, and pigment stem cells. Genetic mutations that disrupt the expression of PROTO-ONCOGENE PROTEINS C-KIT are associated with PIEBALDISM, while overexpression or constitutive activation of the c-kit protein-tyrosine kinase is associated with tumorigenesis.
Granulated cells that are found in almost all tissues, most abundantly in the skin and the gastrointestinal tract. Like the BASOPHILS, mast cells contain large amounts of HISTAMINE and HEPARIN. Unlike basophils, mast cells normally remain in the tissues and do not circulate in the blood. Mast cells, derived from the bone marrow stem cells, are regulated by the STEM CELL FACTOR.
A form of systemic mastocytosis (MASTOCYTOSIS, SYSTEMIC) characterized by the presence of large numbers of tissue MAST CELLS in the peripheral blood without skin lesions. It is a high-grade LEUKEMIA disease with bone marrow smear of >20% MAST CELLS, multi-organ failure and a short survival.
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.
Histamine substituted in any position with one or more methyl groups. Many of these are agonists for the H1, H2, or both histamine receptors.
A family of neutral serine proteases with CHYMOTRYPSIN-like activity. Chymases are primarily found in the SECRETORY GRANULES of MAST CELLS and are released during mast cell degranulation.
Removal of bone marrow and evaluation of its histologic picture.
A parasite of carnivorous mammals that causes TRICHINELLOSIS. It is especially common in rats and in swine fed uncooked garbage. Human infection is initiated by the consumption of raw or insufficiently cooked pork or other meat containing the encysted larvae.
An acute hypersensitivity reaction due to exposure to a previously encountered ANTIGEN. The reaction may include rapidly progressing URTICARIA, respiratory distress, vascular collapse, systemic SHOCK, and death.
A solid tumor consisting of a dense infiltration of MAST CELLS. It is generally benign.
An infection with TRICHINELLA. It is caused by eating raw or undercooked meat that is infected with larvae of nematode worms TRICHINELLA genus. All members of the TRICHINELLA genus can infect human in addition to TRICHINELLA SPIRALIS, the traditional etiological agent. It is distributed throughout much of the world and is re-emerging in some parts as a public health hazard and a food safety problem.
A multifunctional cytokine secreted by primarily by activated TH2 CELLS that may play a role as a regulator of allergic INFLAMMATION. It has been shown to enhance the growth and CELL DIFFERENTIATION of MAST CELLS, and can act on a variety of other immune cells.
Infections of the INTESTINES with PARASITES, commonly involving PARASITIC WORMS. Infections with roundworms (NEMATODE INFECTIONS) and tapeworms (CESTODE INFECTIONS) are also known as HELMINTHIASIS.
A histamine H1 receptor antagonist that is effective in the treatment of chronic urticaria, dermatitis, and histamine-mediated pruritus. Unlike its major metabolite CETIRIZINE, it does cause drowsiness. It is also effective as an antiemetic, for relief of anxiety and tension, and as a sedative.
Physiologically active prostaglandins found in many tissues and organs. They show pressor activity, are mediators of inflammation, and have potential antithrombotic effects.
Infections caused by infestation with worms of the class Trematoda.
Class of parasitic flukes consisting of three subclasses, Monogenea, Aspidogastrea, and Digenea. The digenetic trematodes are the only ones found in man. They are endoparasites and require two hosts to complete their life cycle.
A hematopoietic growth factor and the ligand of the cell surface c-kit protein (PROTO-ONCOGENE PROTEINS C-KIT). It is expressed during embryogenesis and is a growth factor for a number of cell types including the MAST CELLS and the MELANOCYTES in addition to the HEMATOPOIETIC STEM CELLS.
Abnormal increase of EOSINOPHILS in the blood, tissues or organs.
A low affinity interleukin-5 receptor subunit that combines with the CYTOKINE RECEPTOR COMMON BETA SUBUNIT to form a high affinity receptor for INTERLEUKIN-5. Several isoforms of the interleukin-5 receptor alpha subunit exist due to multiple ALTERNATIVE SPLICING.
Venoms from animals of the phylum Arthropoda. Those most investigated are from scorpions and spiders of the class Arachnidae and from ant, bee, and wasp families of the Insecta order Hymenoptera. The venoms contain protein toxins, enzymes, and other bioactive substances and may be lethal to man.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
Pathological processes of the VULVA.

Cutaneous and systemic manifestations of mastocytosis. (1/170)

Mastocytosis is characterized by an excessive number of apparently normal mast cells in the skin and, occasionally, in other organs. Characteristic skin lesions, called urticaria pigmentosa, are present in most patients, but clinical presentation can vary from a pruritic rash to unexplained collapse and sudden death. These lesions are typically tan to red-brown macules that appear on the trunk and spread symmetrically. Patients with mastocytosis often have a long history of chronic and acute symptoms that were unrecognized as mastocytosis. Skin lesions may or may not accompany systemic mastocytosis. Systemic disease may involve the gastrointestinal tract, the bone marrow or other organs. Even when the disease is considered as a possibility by the physician, the diagnosis can be difficult because of special technical requirements necessary for biopsy and because of the problems with biochemical testing. Drug therapy is initiated to stabilize mast cell membranes, to reduce the severity of the attacks and to block the action of inflammatory mediators. The mainstay of therapy is histamine H1 and H2 blockers and the avoidance of triggering factors.  (+info)

Stem cell factor is localized in, released from, and cleaved by human mast cells. (2/170)

Stem cell factor (SCF) is the most important cytokine regulating human mast cell growth and functions. The immunogold technique showed SCF in the secretory granules of skin mast cells and in lung parenchymal mast cells (HLMC). Immunoreactive SCF (iSCF) was detected in cell lysates of HLMC, but not in basophils; iSCF and histamine were detected in supernatants of HLMC 3 min after challenge with anti-FcepsilonRI or anti-IgE, and iSCF in supernatants rapidly declined after 30 min, whereas histamine remained unchanged for 120 min. HPLC and electrospray mass spectrometry (ES/MS) analysis of recombinant human SCF1-166 (18,656. 9 +/- 0.9 Da) treated with chymase showed a polypeptide of 17,977.1 +/- 0.6 Da and a minor component of 697.4 +/- 0.1 Da generated by specific cleavage at Phe159. SCF1-166 and SCF1-159 similarly activated HLMC, potentiated anti-IgE-induced activation of these cells, and stimulated HLMC chemotaxis. SCF159-166 had no effect on mast cells. Western blot analysis of supernatants of anti-IgE-activated HLMC incubated with recombinant human SCF1-166 showed that SCF1-166 was rapidly cleaved to SCF1-159 and SCF1-144. Experiments with supernatants of anti-IgE-activated HLMC incubated with SCF1-166 yielded similar results. In conclusion, SCF is stored in mast cell secretory granules and is immunologically released by human mast cells. SCF1-166 is rapidly and specifically cleaved to SCF1-159 by chymase, which retains its biological effect on mast cells. SCF is also cleaved by other proteases to several SCF species whose possible biological activities remain to be established.  (+info)

An unusual presentation of systemic mastocytosis. (3/170)

A 47 year old man presented with mastocytosis, a disease process characterised by proliferation of mast cells. The clinical features and outcome are discussed.  (+info)

Cutaneous mucinosis and mastocytosis in a shar-pei. (4/170)

A 7-year-old shar-pei was presented because of a recurrent dermatologic condition. Skin biopsies revealed an idiopathic (primary) cutaneous mucinosis that initially responded to corticosteroids. The condition reappeared 2 years later and subsequent biopsies revealed a mast cell tumor in some of the skin sites previously diagnosed with mucinosis.  (+info)

c-Kit and c-kit mutations in mastocytosis and other hematological diseases. (5/170)

Mast cells (MC) are tissue elements derived from hematopoietic stem cells. Their differentiation and proliferation processes are under the influence of cytokines, including one of utmost importance known as stem cell factor (SCF). SCF receptor is encoded by the protooncogene c-kit, belongs to the type III receptor tyrosine kinase subfamily, and is also expressed on other hematopoietic or non-hematopoietic cells. Ligation of c-kit receptor by SCF induces its dimerization, followed by induction of multiple intracellular signaling pathways leading to cell proliferation and activation. Mastocytosis, a relatively rare group of diseases characterized by accumulation of MC in various tissues, are found isolated or sometimes associated with other hematological malignancies in humans. Although the initial events leading to mastocytosis are not yet unraveled, alterations of the c-kit gene have been described. Particularly interesting are acquired mutations resulting in a constitutively activated receptor, possibly involved in the increased numbers of MC in tissues. For this reason, future strategies might be envisaged to target specifically the mutated c-kit and/or its intracellular signaling.  (+info)

A role of mast cell glycosaminoglycans for the immunological expulsion of intestinal nematode, Strongyloides venezuelensis. (6/170)

We examined effects of mast cell glycosaminoglycans on the establishment of the intestinal nematode, Strongyloides venezuelensis, in the mouse small intestine. When intestinal mastocytosis occurred, surgically implanted adult worms could not invade and establish in the intestinal mucosa. In mast cell-deficient W/Wv mice, inhibition of adult worm invasion was not evident as compared with littermate +/+ control mice. Mucosal mastocytosis and inhibition of S. venezuelensis adult worm mucosal invasion was tightly correlated. To determine effector molecules for the invasion inhibition, adult worms were implanted with various sulfated carbohydrates including mast cell glycosaminoglycans. Among sulfated carbohydrates tested, chondroitin sulfate (ChS)-A, ChS-E, heparin, and dextran sulfate inhibited invasion of adult worms into intestinal mucosa in vivo. No significant inhibition was observed with ChS-C, desulfated chondroitin, and dextran. ChS-E, heparin, and dextran sulfate inhibited adhesion of S. venezuelensis adult worms to plastic surfaces in vitro. Furthermore, binding of intestinal epithelial cells to adhesion substances of S. venezuelensis, which have been implicated in mucosal invasion, was inhibited by ChS-E, heparin, and dextran sulfate. Because adult worms of S. venezuelensis were actively moving in the intestinal mucosa, probably exiting and reentering during infection, the possible expulsion mechanism for S. venezuelensis is inhibition by mast cell glycosaminoglycans of attachment and subsequent invasion of adult worms into intestinal epithelium.  (+info)

Nitric oxide mediates intestinal pathology but not immune expulsion during Trichinella spiralis infection in mice. (7/170)

The relationship between intestinal pathology and immune expulsion of gastrointestinal (GI) nematodes remains controversial. Although immune expulsion of GI helminth parasites is usually associated with Th2 responses, the effector mechanisms directly responsible for parasite loss have not been identified. We have previously shown that while the intestinal pathology accompanying the expulsion of the GI parasite Trichinella spiralis may be dependent on IL-4 and mediated by TNF, parasite loss is independent of TNF. In contrast, intestinal pathology in other disease models has been attributed to Th1 cytokines, although it closely resembles that seen in helminth infections. Whereas production of inducible NO synthase (iNOS) in the gut is important for both homeostasis of the epithelial layer and in protection against pathogenic microorganisms, overproduction of NO has been implicated in the pathogenesis of a number of inflammatory conditions. We therefore investigated the role of NO in T. spiralis infection using iNOS-deficient mice. iNOS-/- and iNOS-/+ mice were infected with T. spiralis, and parasite expulsion and intestinal pathology were followed. Parasite expulsion proceeded similarly in both groups of animals, but significant intestinal pathology was only observed in the heterozygous mice. Thus it appears that, although the protective effects of Th2 responses in GI helminth infection do not require NO, this mediator contributes substantially to the associated enteropathy. NO may therefore be an important mediator of enteropathy in both Th1- and Th2-inducing conditions.  (+info)

Pulmonary manifestation of systemic mast cell disease. (8/170)

Systemic mast cell disease is a rare disease of unknown aetiology. Systemic infiltration and proliferation of mast cells in skin, bone marrow, gastrointestinum and lymph nodes is the central pathological feature. This study reports a patient with mastocytosis of the skin (urticaria pigmentosa) for 10 yrs. The patient was referred to hospital for dyspnoea. Chest radiograph showed moderate reticular infiltration of both lungs, computerized tomography revealed multiple lymph nodes of the mediastinum and faint nodular lesions of middle and upper areas of lungs. Transbronchial biopsy demonstrated mast cell infiltration of the lung with formation of mast cell granuloma. According to the current literature, systemic mast cell disease with pulmonary involvement is a very rare entity. After a treatment with interferon alpha-2a over 6 months, the patient's condition and particularly dyspnoea showed improvement in parallel with an amelioration of the lesions as demonstrated by thorax computed tomography.  (+info)

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.

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.

Cutaneous mastocytosis is a condition characterized by the abnormal accumulation of mast cells in the skin. Mast cells are a type of immune cell that releases chemicals such as histamine, heparin, and leukotrienes, which play a role in allergic reactions and inflammation. In cutaneous mastocytosis, the excessive buildup of mast cells can cause various skin symptoms, including redness, itching, swelling, and formation of lesions or tumors.

The condition is typically divided into several subtypes based on the age of onset and the clinical presentation. The most common form in children is called urticaria pigmentosa, which presents as small, reddish-brown spots or bumps that may become raised and itchy when scratched or rubbed (Darier's sign). In adults, a more severe form known as diffuse cutaneous mastocytosis can occur, where the entire skin becomes thickened, red, and swollen.

Cutaneous mastocytosis is usually diagnosed based on the patient's medical history, physical examination, and results from skin biopsies. Treatment typically focuses on relieving symptoms and preventing mast cell activation. Medications such as antihistamines, topical steroids, and mast cell stabilizers may be used to control itching, flushing, and other symptoms associated with the condition. In some cases, systemic therapies or phototherapy may also be recommended.

Urticaria pigmentosa is a rare mast cell disorder, characterized by the development of brownish-red, raised lesions (maculopapules) on the skin. These lesions are often found on the trunk and proximal extremities, but can occur anywhere on the body. They are typically asymptomatic, but may become itchy or even painful when subjected to friction, heat, or emotional stress. In some cases, these lesions may also release histamine, leading to symptoms such as flushing, headache, and hypotension. Urticaria pigmentosa is more common in children than adults, and typically resolves on its own over time. However, in some cases it can persist into adulthood or even progress to systemic mastocytosis, a more severe form of the disorder that can affect internal organs.

Tryptase is a type of enzyme that is found in the cells called mast cells, which are a part of the immune system. Specifically, tryptase is a serine protease, which means it helps to break down other proteins in the body. Tryptase is often released during an allergic reaction or as part of an inflammatory response. It can be measured in the blood and is sometimes used as a marker for mast cell activation or degranulation. High levels of tryptase may indicate the presence of certain medical conditions, such as systemic mastocytosis or anaphylaxis.

Proto-oncogene proteins c-kit, also known as CD117 or stem cell factor receptor, are transmembrane receptor tyrosine kinases that play crucial roles in various biological processes, including cell survival, proliferation, differentiation, and migration. They are encoded by the c-KIT gene located on human chromosome 4q12.

These proteins consist of an extracellular ligand-binding domain, a transmembrane domain, and an intracellular tyrosine kinase domain. The binding of their ligand, stem cell factor (SCF), leads to receptor dimerization, autophosphorylation, and activation of several downstream signaling pathways such as PI3K/AKT, MAPK/ERK, and JAK/STAT.

Abnormal activation or mutation of c-kit proto-oncogene proteins has been implicated in the development and progression of various malignancies, including gastrointestinal stromal tumors (GISTs), acute myeloid leukemia (AML), mast cell diseases, and melanoma. Targeted therapies against c-kit, such as imatinib mesylate (Gleevec), have shown promising results in the treatment of these malignancies.

Mast cells are a type of white blood cell that are found in connective tissues throughout the body, including the skin, respiratory tract, and gastrointestinal tract. They play an important role in the immune system and help to defend the body against pathogens by releasing chemicals such as histamine, heparin, and leukotrienes, which help to attract other immune cells to the site of infection or injury. Mast cells also play a role in allergic reactions, as they release histamine and other chemicals in response to exposure to an allergen, leading to symptoms such as itching, swelling, and redness. They are derived from hematopoietic stem cells in the bone marrow and mature in the tissues where they reside.

Mast cell leukemia is a rare and aggressive type of leukemia, which is a cancer of the white blood cells. Specifically, mast cell leukemia affects a particular type of white blood cell called a mast cell. Mast cells are part of the immune system and play a role in allergic reactions and inflammation.

In mast cell leukemia, the bone marrow produces too many immature mast cells, which then enter the bloodstream. These abnormal mast cells can accumulate in various organs, such as the spleen, liver, and lymph nodes, causing damage and enlargement of these organs.

Symptoms of mast cell leukemia may include fatigue, weight loss, frequent infections, easy bruising or bleeding, bone pain, and enlarged lymph nodes. Diagnosis typically involves blood tests, bone marrow aspiration and biopsy, and imaging studies to assess the extent of organ involvement.

Mast cell leukemia is a very aggressive cancer with a poor prognosis, and treatment options are limited. Current treatments may include chemotherapy, stem cell transplantation, and targeted therapy with drugs that target specific molecular abnormalities in mast cells. However, the response to treatment is often not durable, and the disease can progress rapidly.

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.

Methylhistamines are not a recognized medical term or a specific medical condition. However, the term "methylhistamine" may refer to the metabolic breakdown product of the antihistamine drug, diphenhydramine, which is also known as N-methyldiphenhydramine or dimenhydrinate.

Diphenhydramine is a first-generation antihistamine that works by blocking the action of histamine, a chemical released during an allergic reaction. When diphenhydramine is metabolized in the body, it is converted into several breakdown products, including methylhistamines.

Methylhistamines are not known to have any specific pharmacological activity or clinical significance. However, they can be used as a marker for the presence of diphenhydramine or its metabolism in the body.

Chymases are a type of enzyme that belong to the family of serine proteases. They are found in various tissues and organs, including the heart, lungs, and immune cells called mast cells. Chymases play a role in several physiological and pathological processes, such as inflammation, tissue remodeling, and blood pressure regulation.

One of the most well-known chymases is found in the mast cells and is often referred to as "mast cell chymase." This enzyme can cleave and activate various proteins, including angiotensin I to angiotensin II, a potent vasoconstrictor that increases blood pressure. Chymases have also been implicated in the development of cardiovascular diseases, such as hypertension and heart failure, as well as respiratory diseases like asthma and chronic obstructive pulmonary disease (COPD).

In summary, chymases are a group of serine protease enzymes that play important roles in various physiological and pathological processes, particularly in inflammation, tissue remodeling, and blood pressure regulation.

A bone marrow examination is a medical procedure in which a sample of bone marrow, the spongy tissue inside bones where blood cells are produced, is removed and examined. This test is used to diagnose or monitor various conditions affecting blood cell production, such as infections, leukemia, anemia, and other disorders of the bone marrow.

The sample is typically taken from the hipbone (iliac crest) or breastbone (sternum) using a special needle. The procedure may be done under local anesthesia or with sedation to minimize discomfort. Once the sample is obtained, it is examined under a microscope for the presence of abnormal cells, changes in cell size and shape, and other characteristics that can help diagnose specific conditions. Various stains, cultures, and other tests may also be performed on the sample to provide additional information.

Bone marrow examination is an important diagnostic tool in hematology and oncology, as it allows for a detailed assessment of blood cell production and can help guide treatment decisions for patients with various blood disorders.

"Trichinella spiralis" is a species of parasitic roundworm that causes the disease trichinosis in humans. The adult worms live in the intestine, where they produce larvae that migrate to striated muscle tissue, including the diaphragm, tongue, and skeletal muscles, where they encyst and form nurse cells. Infection typically occurs through the consumption of undercooked or raw meat, particularly pork, contaminated with the larvae. Symptoms can range from gastrointestinal disturbances to fever, muscle pain, and potentially life-threatening complications in severe cases. Prevention includes cooking meat thoroughly and freezing it at certain temperatures to kill the larvae.

Anaphylaxis is a severe, life-threatening systemic allergic reaction that occurs suddenly after exposure to an allergen (a substance that triggers an allergic reaction) to which the person has previously been sensitized. The symptoms of anaphylaxis include rapid onset of symptoms such as itching, hives, swelling of the throat and tongue, difficulty breathing, wheezing, cough, chest tightness, rapid heartbeat, hypotension (low blood pressure), shock, and in severe cases, loss of consciousness and death. Anaphylaxis is a medical emergency that requires immediate treatment with epinephrine (adrenaline) and other supportive measures to stabilize the patient's condition.

A mastocytoma is a type of tumor that develops from mast cells, which are a part of the immune system and play a role in allergic reactions and inflammation. Mastocytomas are most commonly found in the skin, but they can also occur in other organs such as the liver, spleen, and lymph nodes.

Mastocytomas are usually benign (non-cancerous), although malignant (cancerous) forms known as mast cell sarcomas can also occur. They typically appear as raised, red or brown lesions on the skin that may be itchy, painful, or bleed easily.

The diagnosis of a mastocytoma is usually made through a biopsy of the tumor, which involves removing a small sample of tissue for examination under a microscope. Treatment options for mastocytomas may include surgical removal, medication to manage symptoms such as itching or flushing, and in some cases, chemotherapy or radiation therapy.

Trichinellosis is a parasitic disease caused by the roundworm Trichinella spiralis. The infection typically occurs when contaminated raw or undercooked meat, often pork, is consumed. After ingestion, the larvae of the worm are released from the cysts in the meat and migrate to the small intestine, where they mature into adults.

The adult females then lay new larvae that penetrate the intestinal wall and travel through the bloodstream to striated muscle tissue (such as skeletal muscles), where they encapsulate and form new cysts. The symptoms of trichinellosis can vary widely, depending on the number of worms ingested and the intensity of infection. Early symptoms may include diarrhea, abdominal pain, nausea, vomiting, and fever. As the larvae migrate to muscle tissue, additional symptoms such as muscle pain, weakness, swelling of the face, eyelids, or tongue, and skin rashes can occur. Severe infections may lead to life-threatening complications, including heart and respiratory failure.

Prevention measures include cooking meat thoroughly (to an internal temperature of at least 160°F or 71°C), freezing meat properly (at -15°F or -26°C for several days) to kill the parasites, and avoiding consumption of raw or undercooked meat, especially from wild animals.

Interleukin-9 (IL-9) is a type of cytokine, which are small signaling proteins that mediate and regulate immunity, inflammation, and hematopoiesis. IL-9 is produced by several types of immune cells, including T cells (a type of white blood cell), mast cells, and eosinophils.

IL-9 plays a role in the development and function of various immune cells, and has been implicated in the pathogenesis of several inflammatory and allergic diseases, such as asthma, atopic dermatitis, and food allergy. It can promote the growth and survival of certain types of immune cells, including mast cells and B cells (another type of white blood cell), and can also enhance their activation and effector functions.

In addition to its role in immunity and inflammation, IL-9 has been shown to play a role in the development and progression of some types of cancer, such as lung cancer and leukemia. However, more research is needed to fully understand the complex functions of this cytokine and its potential as a therapeutic target.

Parasitic intestinal diseases are disorders caused by microscopic parasites that invade the gastrointestinal tract, specifically the small intestine. These parasites include protozoa (single-celled organisms) and helminths (parasitic worms). The most common protozoan parasites that cause intestinal disease are Giardia lamblia, Cryptosporidium parvum, and Entamoeba histolytica. Common helminthic parasites include roundworms (Ascaris lumbricoides), tapeworms (Taenia saginata and Taenia solium), hookworms (Ancylostoma duodenale and Necator americanus), and pinworms (Enterobius vermicularis).

Parasitic intestinal diseases can cause a variety of symptoms, including diarrhea, abdominal pain, bloating, nausea, vomiting, fatigue, and weight loss. The severity and duration of the symptoms depend on the type of parasite, the number of organisms present, and the immune status of the host.

Transmission of these parasites can occur through various routes, including contaminated food and water, person-to-person contact, and contact with contaminated soil or feces. Preventive measures include practicing good hygiene, washing hands thoroughly after using the toilet and before handling food, cooking food thoroughly, and avoiding consumption of raw or undercooked meat, poultry, or seafood.

Treatment of parasitic intestinal diseases typically involves the use of antiparasitic medications that target the specific parasite causing the infection. In some cases, supportive care such as fluid replacement and symptom management may also be necessary.

Hydroxyzine is an antihistamine medication that is primarily used to treat symptoms of allergies such as itching, hives, and swelling. It works by blocking the effects of histamine, a substance in the body that causes allergic reactions. In addition to its antihistaminic properties, hydroxyzine also has sedative and anxiety-reducing effects, which make it useful in treating anxiety disorders, symptoms of alcohol withdrawal, and as a sleep aid. It is available in both oral and injectable forms and is usually taken orally in the form of tablets, capsules, or syrup. As with any medication, hydroxyzine should be used under the supervision of a healthcare provider, and its use may be subject to certain precautions and contraindications depending on the individual's medical history and current health status.

I'm sorry for any confusion, but "Prostaglandins D" is not a recognized or established term in the field of medicine or physiology. Prostaglandins are a group of lipid compounds that are synthesized in the body from fatty acids and have diverse hormone-like effects in the body. They are classified into several types based on their chemical structure and biological activity, including Prostaglandin D (PGD), but referring to "Prostaglandins D" as a single entity is not standard.

Prostaglandin D (PGD) itself is a type of prostaglandin that is rapidly converted to other compounds in the body and has been studied for its potential role in various physiological processes, such as inflammation, fever, and blood flow regulation. However, it's important to note that specific medical definitions or clinical uses related to "Prostaglandins D" are not well-established.

If you have any further questions or need more information about a specific aspect of prostaglandins or their role in the body, I would be happy to help!

Trematode infections, also known as trematodiasis or fluke infections, are parasitic diseases caused by various species of flatworms called trematodes. These parasites have an indirect life cycle involving one or two intermediate hosts (such as snails or fish) and a definitive host (usually a mammal or bird).

Humans can become accidentally infected when they consume raw or undercooked aquatic plants, animals, or contaminated water that contains the larval stages of these parasites. The most common trematode infections affecting humans include:

1. Schistosomiasis (also known as bilharzia): Caused by several species of blood flukes (Schistosoma spp.). Adult worms live in the blood vessels, and their eggs can cause inflammation and damage to various organs, such as the liver, intestines, bladder, or lungs.
2. Liver flukes: Fasciola hepatica and Fasciola gigantica are common liver fluke species that infect humans through contaminated watercress or other aquatic plants. These parasites can cause liver damage, abdominal pain, diarrhea, and eosinophilia (elevated eosinophil count in the blood).
3. Lung flukes: Paragonimus spp. are lung fluke species that infect humans through consumption of raw or undercooked crustaceans. These parasites can cause coughing, chest pain, and bloody sputum.
4. Intestinal flukes: Various species of intestinal flukes (e.g., Haplorchis spp., Metagonimus yokogawai) infect humans through consumption of raw or undercooked fish. These parasites can cause abdominal pain, diarrhea, and eosinophilia.
5. Eye fluke: The oriental eye fluke (Drepanotrema spp.) can infect the human eye through contaminated water. It can cause eye inflammation, corneal ulcers, and vision loss.

Prevention measures include avoiding consumption of raw or undercooked aquatic plants, animals, and their products; practicing good hygiene; and treating drinking water to kill parasites. Treatment typically involves administering anthelmintic drugs such as praziquantel, albendazole, or mebendazole, depending on the specific fluke species involved.

Trematoda is a class of parasitic flatworms, also known as flukes. They have a complex life cycle involving one or more intermediate hosts and a definitive host. Adult trematodes are typically leaf-shaped and range in size from a few millimeters to several centimeters.

They have a characteristic oral sucker surrounding the mouth and a ventral sucker, which they use for locomotion and attachment to their host's tissues. Trematodes infect various organs of their hosts, including the liver, lungs, blood vessels, and intestines, causing a range of diseases in humans and animals.

Examples of human-infecting trematodes include Schistosoma spp., which cause schistosomiasis (also known as bilharzia), and Fasciola hepatica, which causes fascioliasis (liver fluke disease). Trematode infections are typically treated with antiparasitic drugs.

Stem Cell Factor (SCF), also known as Kit Ligand or Steel Factor, is a growth factor that plays a crucial role in the regulation of hematopoiesis, which is the process of producing various blood cells. It is a glycoprotein that binds to the c-Kit receptor found on hematopoietic stem cells and progenitor cells, promoting their survival, proliferation, and differentiation into mature blood cells.

SCF is involved in the development and function of several types of blood cells, including red blood cells, white blood cells, and platelets. It also plays a role in the maintenance and self-renewal of hematopoietic stem cells, which are essential for the continuous production of new blood cells throughout an individual's lifetime.

In addition to its role in hematopoiesis, SCF has been implicated in various other biological processes, such as melanogenesis, gametogenesis, and tissue repair and regeneration. Dysregulation of SCF signaling has been associated with several diseases, including certain types of cancer, bone marrow failure disorders, and autoimmune diseases.

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.

The Interleukin-5 Receptor alpha Subunit (IL-5Rα) is a protein that forms part of the Type I cytokine receptor, specifically for the interleukin-5 (IL-5) cytokine. This receptor is found on the surface of hematopoietic cells, such as eosinophils and basophils. The binding of IL-5 to the IL-5Rα subunit initiates intracellular signaling cascades that regulate the growth, activation, differentiation, and survival of eosinophils and basophils, which are crucial in the immune response against parasitic infections and allergic reactions. Mutations in the gene encoding IL-5Rα can lead to altered immune responses and diseases such as hypereosinophilic syndromes.

Arthropod venoms are toxic secretions produced by the venom glands of various arthropods, such as spiders, scorpions, insects, and marine invertebrates. These venoms typically contain a complex mixture of bioactive molecules, including peptides, proteins, enzymes, and small molecules, which can cause a range of symptoms and effects in humans and other animals.

The specific composition of arthropod venoms varies widely depending on the species and can be tailored to serve various functions, such as prey immobilization, defense, or predation. Some arthropod venoms contain neurotoxins that can disrupt nerve function and cause paralysis, while others may contain cytotoxins that damage tissues or hemotoxins that affect the blood and cardiovascular system.

Arthropod venoms have been studied for their potential therapeutic applications, as some of their bioactive components have shown promise in treating various medical conditions, including pain, inflammation, and neurological disorders. However, it is important to note that arthropod venoms can also cause severe allergic reactions and other adverse effects in susceptible individuals, making it essential to exercise caution when handling or coming into contact with venomous arthropods.

A biopsy is a medical procedure in which a small sample of tissue is taken from the body to be examined under a microscope for the presence of disease. This can help doctors diagnose and monitor various medical conditions, such as cancer, infections, or autoimmune disorders. The type of biopsy performed will depend on the location and nature of the suspected condition. Some common types of biopsies include:

1. Incisional biopsy: In this procedure, a surgeon removes a piece of tissue from an abnormal area using a scalpel or other surgical instrument. This type of biopsy is often used when the lesion is too large to be removed entirely during the initial biopsy.

2. Excisional biopsy: An excisional biopsy involves removing the entire abnormal area, along with a margin of healthy tissue surrounding it. This technique is typically employed for smaller lesions or when cancer is suspected.

3. Needle biopsy: A needle biopsy uses a thin, hollow needle to extract cells or fluid from the body. There are two main types of needle biopsies: fine-needle aspiration (FNA) and core needle biopsy. FNA extracts loose cells, while a core needle biopsy removes a small piece of tissue.

4. Punch biopsy: In a punch biopsy, a round, sharp tool is used to remove a small cylindrical sample of skin tissue. This type of biopsy is often used for evaluating rashes or other skin abnormalities.

5. Shave biopsy: During a shave biopsy, a thin slice of tissue is removed from the surface of the skin using a sharp razor-like instrument. This technique is typically used for superficial lesions or growths on the skin.

After the biopsy sample has been collected, it is sent to a laboratory where a pathologist will examine the tissue under a microscope and provide a diagnosis based on their findings. The results of the biopsy can help guide further treatment decisions and determine the best course of action for managing the patient's condition.

Vulvar diseases refer to a range of medical conditions that affect the vulva, which is the external female genital area including the mons pubis, labia majora and minora, clitoris, and the vaginal opening. These conditions can cause various symptoms such as itching, burning, pain, soreness, irritation, or abnormal growths or lesions. Some common vulvar diseases include:

1. Vulvitis: inflammation of the vulva that can be caused by infection, allergies, or irritants.
2. Lichen sclerosus: a chronic skin condition that causes thin, white patches on the vulva.
3. Lichen planus: an inflammatory condition that affects the skin and mucous membranes, including the vulva.
4. Vulvar cancer: a rare type of cancer that develops in the tissues of the vulva.
5. Genital warts: caused by human papillomavirus (HPV) infection, these are small growths or bumps on the vulva.
6. Pudendal neuralgia: a nerve condition that causes pain in the vulvar area.
7. Vestibulodynia: pain or discomfort in the vestibule, the area surrounding the vaginal opening.

It is important to consult a healthcare professional if experiencing any symptoms related to vulvar diseases for proper diagnosis and treatment.

Find symptoms and other information about Systemic mastocytosis. ... Systemic mastocytosis (SM) is a form of mastocytosis in which ... Systemic mastocytosis (SM) is a form of mastocytosis in which mast cells accumulate in internal tissues and organs such as the ... Familial cases rarely have been reported.Systemic mastocytosis (SM) is a form of mastocytosis in which mast cells accumulate in ... When Do Symptoms of Systemic mastocytosis Begin?. Symptoms of this disease may start to appear at any time in life.. The age ...
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Treatment of Mastocytosis A standard treatment for mastocytosis is difficult to apply to individual patients based on the ... Table 1. Table 1. Mastocytosis Classification System Year 2000 Working Conference on Mastocytosis ... Aspirin has been reported to cause death in one patient with mastocytosis (Oklahoma: An Educational Success, 2002). Mast cell ... or cetirizine may be used to treat symptoms of mastocytosis. Diphenhydramine, hydroxyzine, and doxepin have been used for ...
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Mastocytosis may remain undiagnosed when it is actually present, or it may be diagnosed even though morphological and molecular ... Because some of the manifestations of mastocytosis are nonspecific and can be mimicked by other diseases, there is a risk of ... Results: The clinical manifestations of mastocytosis range from cutaneous mastocytosis, a common, prognostically favorable ... Mastocytosis: a disease of the hematopoietic stem cell Dtsch Arztebl Int. 2008 Oct;105(40):686-92. doi: 10.3238/arztebl. ...
The following factors may raise a persons risk of developing mastocytosis:. * Age. Most often, cutaneous mastocytosis begins ... Changed or mutated forms of the c-kit gene may cause some types of mastocytosis, including systemic mastocytosis. ... Mastocytosis: Risk Factors. Approved by the Cancer.Net Editorial Board, 06/2017 ... Some people with several risk factors never develop mastocytosis, while others with no known risk factors do. Knowing your risk ...
Does anybody have systemic mastocytosis that has caused a sarcoma in their Liver. Or does anyone know about undifferentiated ... Does anybody have systemic mastocytosis that has caused a sarcoma in their Liver. Or does anyone know about undifferentiated ...
Mastocytosis is a rare and underdiagnosed disorder characterized by mast cell proliferation in the tissues and organs of the ... Gastrointestinal manifestations of systemic mastocytosis. Lee JK, Whittaker SJ, Enns RA, Zetler P. Lee JK, et al. World J ... Mastocytosis is a rare and underdiagnosed disorder characterized by mast cell proliferation in the tissues and organs of the ... Immunoreactivity for CD25 in gastrointestinal mucosal mast cells is specific for systemic mastocytosis. Hahn HP, Hornick JL. ...
... presents the case of a patient with indolent systemic mastocytosis and urticaria pigmentosa. ... Systemic Mastocytosis : Episode 12. Case 3: Indolent Systemic Mastocytosis September 7, 2021. Daniel J. DeAngelo, MD, PhD, Dana ... Desensitization in mastocytosis is certainly not as successful as somebody without mastocytosis. I had mentioned we sometimes ... Case 3: Indolent Systemic Mastocytosis EP: 13. .Case 4: A 68-Year-Old Female With Mast Cell Leukemia EP: 14. .Case 4: Treatment ...
Mastocytosis is characterized by large amounts of mast cells (MCs) accumulating in the body. Abnormal activation of MCs leads ... Two major types are cutaneous (CM) and systemic (SM) mastocytosis. There are several types of CM, including maculopapular (MPCM ... Mastocytosis is divided into 7 categories based on World Health Organization classification. ... Dharshana Krishnaprasadh, Kelly Levasseur; Mastocytosis. Pediatr Rev August 2021; 42 (8): 468-470. https://doi.org/10.1542/PIR. ...
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systemic mastocytosis; acute myeloid leukaemia; mastocytosis; haematological neoplasm Abstract. Bacground: The pathogenesis of ... Systemic mastocytosis associated with acute myeloid leukaemia Authors. * Mohd Nasiruddin Dhamirah Nazihah Department of ... Due to an extensive accumulation of AML blast cells that obscured the mast cell infiltrates, mastocytosis was not evident at ... and concurrent mastocytosis according to World Health Organization (WHO) classification. ...
Usefulness of radiological and biochemical examinations in diagnosis of systemic involvement in patients with skin mastocytosis ...
The standard treatment in mastocytosis is anti-mediator treatment; however, sometimes this is not enough. Find out what you ... Non-advanced mastocytosis is the most common form of mastocytosis, and is generally associated with a normal or a near-normal ... Rationale for Tyrosine Kinase Inhibitors in Non-Advanced Systemic Mastocytosis. Be part of the knowledge.â„¢. *Log In ... And when you compare the symptom burden and systemic mastocytosis to other diagnoses, we see that this is looking at SF 12 ...
Mastocytosis - Learn about the causes, symptoms, diagnosis & treatment from the MSD Manuals - Medical Consumer Version. ... Systemic mastocytosis may affect bone marrow, and up to 30% of adults with systemic mastocytosis develop cancers, particularly ... Mastocytosis (Mast Cell Disease). By James Fernandez , MD, PhD, Cleveland Clinic Lerner College of Medicine at Case Western ... If mastocytosis affects only the skin, it may resolve without treatment, but if it affects other parts of the body, it cannot ...
... systemic mastocytosis with associated hematological neoplasm (SM-AHN), or mast cell leukemia (MCL). Approval was based on resu ... granted regular approval to midostaurin for the treatment of adult patients with aggressive systemic mastocytosis (ASM), ... FDA Approval Summary: Midostaurin for the Treatment of Advanced Systemic Mastocytosis Yvette L Kasamon 1 , Chia-Wen Ko 2 , ... FDA Approval Summary: Midostaurin for the Treatment of Advanced Systemic Mastocytosis Yvette L Kasamon et al. Oncologist. 2018 ...
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Systemic mastocytosis is widely underdiagnosed, and many more hematologic oncologists should be looking for it, say experts. ... ICC 2022 divides advanced systemic mastocytosis into three subtypes: aggressive systemic mastocytosis (ASM), systemic ... of systemic mastocytosis cases to be indolent disease, the incidence of advanced systemic mastocytosis in the United States ... More than 80% of systemic mastocytosis is driven by the KIT D816V mutation. Along with a host of other rare KIT mutations, KIT ...
Urticaria Pigmentosa is a benign type of cutaneous mastocytosis that I occasionally encounter in both my The Woodlands ... Urticaria Pigmentosa is a benign type of cutaneous mastocytosis that I occasionally encounter in both my The Woodlands ... Urticaria Pigmentosa accounts for the majority of childhood mastocytosis cases. Clinically, Urticaria Pigmentosa appears as ...
MPCM: maculopapular cutaneous mastocytosis; CM: cutaneous mastocytosis.. Reproduced from: Hartmann K, Escribano L, Grattan C, ... Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; ... MPCM in patients with childhood-onset mastocytosis.. (A-G) Most children have characteristic large, brown lesions of different ...
Systemic Mastocytosis Variants, including B and C findings and Mast Cell Leukemia ...
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The Phase III PIONEER study in indolent systemic mastocytosis met the primary endpoint, but the magnitude of benefit came up ... Blueprint Prepares To Take On Novartis In Advanced Systemic Mastocytosis The companys rollout plans for Ayvakit appear similar ... The Phase III PIONEER study in indolent systemic mastocytosis met the primary endpoint, but the magnitude of benefit came up ... Source: Shutterstock Blueprint Medicines announced Phase III data for Ayvakit in indolent systemic mastocytosis ...
  • Diagnosis of urticaria pigmentosa (cutaneous mastocytosis, see above) can often be done by seeing the characteristic lesions that are dark brown and fixed. (wikipedia.org)
  • Mastocytosis can occur in a variety of forms: The most common cutaneous mastocytosis is maculopapular cutaneous mastocytosis, previously named papular urticaria pigmentosa (UP), more common in children, although also seen in adults. (wikipedia.org)
  • Telangiectasia macularis eruptiva perstans (TMEP) is a much rarer form of cutaneous mastocytosis that affects adults. (wikipedia.org)
  • Genotype and phenotype analysis of patients with pediatric cutaneous mastocytosis, especially wild-type KIT patients. (medscape.com)
  • Macri A, Cook C. Urticaria Pigmentosa (Cutaneous Mastocytosis). (medscape.com)
  • Expression of Bcl-2 and Bcl-xL in cutaneous and bone marrow lesions of mastocytosis. (medscape.com)
  • Soilleux EJ, Brown VL, Bowling J. Cutaneous mastocytosis localized to a radiotherapy field. (medscape.com)
  • Cutaneous mastocytosis in human immunodeficiency virus: an unfortunate coincidence? (medscape.com)
  • The clinical manifestations of mastocytosis range from cutaneous mastocytosis, a common, prognostically favorable presentation, to mast cell leukemia, a rare, life-threatening disease. (nih.gov)
  • Most often, cutaneous mastocytosis begins during infancy and early childhood. (cancer.net)
  • A skin biopsy confirmed urticaria pigmentosa, or cutaneous mastocytosis. (onclive.com)
  • Two major types are cutaneous (CM) and systemic (SM) mastocytosis. (aap.org)
  • Cutaneous mastocytosis usually occurs in children. (msdmanuals.com)
  • Urticaria Pigmentosa is a benign type of cutaneous mastocytosis that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. (perridermatology.com)
  • According to mast cells localization, it is possible to discriminate cutaneous mastocytosis (CM) from systemic mastocytosis (SM), the latter involving at least an extracutaneous organ, like bone marrow, liver, spleen and gastrointestinal tract. (unica.it)
  • [ 1 ] Mastocytosis encompasses cutaneous mastocytosis and systemic mastocytosis. (cosmoderma.org)
  • Cutaneous mastocytosis can present in different forms such as maculopapular, diffuse, or mastocytoma. (cosmoderma.org)
  • [ 2 ] Bullous mastocytosis is a rare variant of diffuse cutaneous mastocytosis and is caused by mast cell degranulation, which releases proteases that separate the dermoepidermal layer. (cosmoderma.org)
  • A 13-year-old boy with cutaneous mastocytosis and type 1 diabetes mellitus developed multiple itchy papules 2 to 3 days after he started receiving insulin (lispro) pump therapy. (aai.org.tr)
  • Herein, we present for the first time, a patient with type 1 diabetes mellitus and cutaneous mastocytosis who developed exacerbation of lesions during insulin pump therapy. (aai.org.tr)
  • Cutaneous biopsy demonstrated diffuse cutaneous mastocytosis. (karger.com)
  • 1. Cutaneous mastocytosis with atypical mast cells and giant cytoplasmic granules. (nih.gov)
  • 2. A case of cutaneous mastocytosis in a child with prominent Langerhans cell infiltration. (nih.gov)
  • 7. Co-localization of tryptase and cathepsin-G in mast cells in cutaneous mastocytosis. (nih.gov)
  • 10. Cutaneous Mastocytosis With Atypical Mast Cells in a 7-Year-Old Girl. (nih.gov)
  • 13. Cutaneous Mastocytosis With Predominant Infiltration of Promastocytes. (nih.gov)
  • 14. Characterization of cellular dermal infiltrates in human cutaneous mastocytosis. (nih.gov)
  • 15. Serum tryptase and SCORMA (SCORing MAstocytosis) Index as disease severity parameters in childhood and adult cutaneous mastocytosis. (nih.gov)
  • 16. Neonatal onset diffuse cutaneous mastocytosis: a case report and review of the literature. (nih.gov)
  • Urticaria pigmentosa is the most common cutaneous manifestation of mastocytosis. (clinicaladvisor.com)
  • A greater understanding of the role of mast cells in innate and acquired immunity has been conducted through the evaluation, characterization and treatment of patients with cutaneous and systemic mastocytosis, a clonal mast cell disorder of excessive mast cell proliferation and activation. (nih.gov)
  • 2] MPCM and TMEP can be a part of indolent systemic mastocytosis. (wikipedia.org)
  • The mildest forms of systemic mastocytosis are the indolent and smoldering types. (medlineplus.gov)
  • Individuals with smoldering mastocytosis may have more organs affected and more severe features than those with indolent mastocytosis. (medlineplus.gov)
  • The indolent type is the most common type of systemic mastocytosis. (medlineplus.gov)
  • Evolution of urticaria pigmentosa into indolent systemic mastocytosis: abnormal immunophenotype of mast cells without evidence of c-kit mutation ASP-816-VAL. (medscape.com)
  • Dan DeAngelo, MD, PhD, presents the case of a patient with indolent systemic mastocytosis and urticaria pigmentosa. (onclive.com)
  • She does have systemic mastocytosis, but she has no C-findings, so it's indolent. (onclive.com)
  • And as I mentioned, they are also being employed in indolent systemic mastocytosis refractory to symptomatic treatments. (reachmd.com)
  • The Phase III PIONEER study in indolent systemic mastocytosis met the primary endpoint, but the magnitude of benefit came up short of analysts' expectations. (citeline.com)
  • Detailed data in indolent systemic mastocytosis show improvement across primary and key secondary endpoints, but analysts pointed to lower improvement on symptoms outside of those affecting the skin. (citeline.com)
  • A speedy FDA review of the approval application for avapritinib for indolent systemic mastocytosis has commenced. (targetedonc.com)
  • The FDA has granted priority review to a supplemental new drug application (sNDA) for avapritinib (AYVAKIT), a potential treatment option for adult patients with indolent systemic mastocytosis. (targetedonc.com)
  • People with indolent systemic mastocytosis experience debilitating symptoms and poor quality of life, and we have the potential to transform clinical outcomes for these patients by targeting the genetic driver of disease with [avapritinib],' said Becker Hewes, MD, chief medical officer at Blueprint Medicines, in a press release. (targetedonc.com)
  • The speedy review of the sNDA comes after avapritinib was granted breakthrough therapy designation by the FDA for the treatment of moderate to severe indolent systemic mastocytosis. (targetedonc.com)
  • 1. Blueprint Medicines announces FDA Acceptance of supplemental new drug application for AYVAKIT® (avapritinib) for the treatment of indolent systemic mastocytosis. (targetedonc.com)
  • Jeff was first diagnosed with systemic mastocytosis early in life, at age 16. (patientworthy.com)
  • Nationwide, approximately 1,000 adults are diagnosed with systemic mastocytosis annually. (medscape.com)
  • Sixteen-year-old Tayla was diagnosed with systemic mastocytosis as a baby, however it took a further four years of specialist appointments, testing and emergency department visits for treatment to begin. (rch.org.au)
  • Many individuals with systemic mastocytosis develop a skin condition called urticaria pigmentosa, which is characterized by raised patches of brownish skin that sting or itch with contact or changes in temperature. (medlineplus.gov)
  • Lappe U, Aumann V, Mittler U, Gollnick H. Familial urticaria pigmentosa associated with thrombocytosis as the initial symptom of systemic mastocytosis and Down's syndrome. (medscape.com)
  • Urticaria pigmentosa rarely progresses to systemic mastocytosis in children but may do so more often in adults. (msdmanuals.com)
  • Urticaria Pigmentosa accounts for the majority of childhood mastocytosis cases. (perridermatology.com)
  • The severe types include aggressive systemic mastocytosis, systemic mastocytosis with an associated hematologic neoplasm, and mast cell leukemia. (medlineplus.gov)
  • Systemic mastocytosis with an associated hematologic neoplasm and mast cell leukemia both involve blood cell disorders or blood cell cancer ( leukemia ). (medlineplus.gov)
  • The pathogenesis of systemic mastocytosis with associated haematological neoplasm (SMAHN) is not well understood. (banglajol.info)
  • In April 2017, the U.S. Food and Drug Administration granted regular approval to midostaurin for the treatment of adult patients with aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated hematological neoplasm (SM-AHN), or mast cell leukemia (MCL). (nih.gov)
  • ICC 2022 divides advanced systemic mastocytosis into three subtypes: aggressive systemic mastocytosis (ASM), systemic mastocytosis with an associated hematologic neoplasm (SM-AHN), and mast cell leukemia (MCL). (medscape.com)
  • Mastocytosis is a myeloproliferative neoplasm characterized by infiltration of clonally derived mast cells in different tissues. (unica.it)
  • Aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated hematological neoplasm (SM-AHN), or mast cell leukemia (MCL). (nih.gov)
  • RYDAPT is indicated for the treatment of adult patients with aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated hematological neoplasm (SM-AHN), or mast cell leukemia (MCL). (nih.gov)
  • RYDAPT is an oral prescription medicine used to treat adults with aggressive systemic mastocytosis (ASM), systemic mastocytosis with associated hematological neoplasm (SM-AHN), or mast cell leukemia (MCL). (rydapt.com)
  • Mutations of the gene coding for the c-kit receptor (mutation KIT(D816V)), leading to constitutive signalling through the receptor is found in >90% of patients with systemic mastocytosis. (wikipedia.org)
  • A standard treatment for mastocytosis is difficult to apply to individual patients based on the variable clinical course. (medscape.com)
  • IL-6 levels predict disease variant and extent of organ involvement in patients with mastocytosis. (medscape.com)
  • As the prevalence of gastrointestinal symptomatology in those patients who have been diagnosed with mastocytosis is so significant, it is an important and relevant disease of which gastroenterology nurses should be cognizant. (nih.gov)
  • Non-advanced systemic mastocytosis is a lifelong disease with severe physical, emotional and social impacts that profoundly reduce patients' quality of life," Lauren Denton, executive director of The Mast Cell Disease Society, said. (pharmalive.com)
  • However, there are many good reasons to identify patients with systemic mastocytosis, according to Attilio Orazi, MD, professor and chair of the department of pathology at Texas Tech University, El Paso. (medscape.com)
  • The second key reason to increase awareness of mastocytosis among physicians, said Dr. Orazi, is that patients falling through the net are likely to be ambulatory, and their presentation can be "a little confusing. (medscape.com)
  • MPCM in patients with childhood-onset mastocytosis. (medilib.ir)
  • 2. Blueprint medicines announces positive top-line results from PIONEER trial of ayvakit® (avapritinib) in patients with non-advanced systemic mastocytosis achieving primary and all key secondary endpoints. (targetedonc.com)
  • Imatinib in systemic mastocytosis: a phase IV clinical trial in patients lacking exon 17 KIT mutations and review of the literature. (qxmd.com)
  • Several factors are known to induce symptoms in patients with mastocytosis. (aai.org.tr)
  • RYDAPT ® (midostaurin) capsules is the first FDA-approved treatment for advanced systemic mastocytosis (SM), including those patients with a certain mutation known as the KIT D816V mutation. (rydapt.com)
  • Patients up to 80 years of age with mastocytosis may be eligible for this 1-day study. (nih.gov)
  • 11. Are gastrointestinal mucosal mast cells increased in patients with systemic mastocytosis? (nih.gov)
  • Skewed Lymphocyte Subpopulations and Associated Phenotypes in Patients with Mastocytosis. (nih.gov)
  • Systemic mastocytosis (SM) is a form of mastocytosis in which mast cells accumulate in internal tissues and organs such as the liver, spleen, bone marrow, and small intestines. (nih.gov)
  • Non-advanced mastocytosis is the most common form of mastocytosis, and is generally associated with a normal or a near-normal life expectancy. (reachmd.com)
  • Interferon alpha-2b (aggressive forms of mastocytosis). (medscape.com)
  • Therapeutic standards for cytoreduction in the progressive, systemic forms of mastocytosis are still lacking. (nih.gov)
  • The results on the acid-soluble fraction of saliva and the preliminary results on the SIC-D complex are promising in order to find candidate markers able to discriminate the different forms of mastocytosis. (unica.it)
  • Changed or mutated forms of the c-kit gene may cause some types of mastocytosis, including systemic mastocytosis. (cancer.net)
  • This photo shows reddish-brown spots on the back of a school-aged child with mastocytosis. (msdmanuals.com)
  • Dental treatment of a Seven-Year-Old Child with Mastocytosis. (bvsalud.org)
  • Since 2001, the diagnosis of mastocytosis has been guided by the World Health Organization Classification of Tumours , or "Blue Book. (medscape.com)
  • When Do Symptoms of Systemic mastocytosis Begin? (nih.gov)
  • Individuals with these types tend to have only the general signs and symptoms of systemic mastocytosis described above. (medlineplus.gov)
  • In cases of suspicion of SM help can also be drawn from analysis of mutation in KIT(D816V) in peripheral blood using sensitive PCR-technology[citation needed] To set the diagnosis of systemic mastocytosis, certain criteria must be met. (wikipedia.org)
  • In most cases of systemic mastocytosis, the accumulated mast cells have a mutation in a gene called KIT . (medlineplus.gov)
  • Tyrosine kinase inhibitors work by inhibiting the disease driving mutation, D816V KIT in mastocytosis, and they can be separated into three broad categories: wild-type KIT inhibitors, and the prototype here is imatinib. (reachmd.com)
  • Some people have a genetic mutation that causes mastocytosis. (msdmanuals.com)
  • More than 80% of systemic mastocytosis is driven by the KIT D816V mutation. (medscape.com)
  • Cytogenetic abnormalities and their lack of relationship to the Asp816Val c-kit mutation in the pathogenesis of mastocytosis. (clinicaladvisor.com)
  • Get updates from the International Mastocytosis and Mast Cell Diseases Awareness Day committee. (mastocytosis-mcas.org)
  • Thank you to Tayla and Leisa for sharing their experience with us on International Mastocytosis and Mast Cell Disease Awareness Day. (rch.org.au)
  • The term "mastocytosis" should not be used in lieu of the diagnosis of mast cell infiltration. (nih.gov)
  • Delineation of patterns of bone marrow mast cell infiltration in systemic mastocytosis: Value of CD25, correlation with subvariations of the disease and separation from mast cell hyperplasia. (nih.gov)
  • Nearly half of individuals with systemic mastocytosis will experience severe allergic reactions (anaphylaxis). (medlineplus.gov)
  • If the base level of s-tryptase is elevated, this implies that the mastocytosis can be systemic. (wikipedia.org)
  • Chemotherapy (aggressive systemic mastocytosis). (medscape.com)
  • Aggressive systemic mastocytosis is associated with a loss of bone tissue (osteoporosis and osteopenia) and multiple bone fractures. (medlineplus.gov)
  • Blueprint Medicines ' Ayvakit (avapritinib) is headed to the FDA for a new indication after the drug hit the mark in Part 2 of the PIONEER trial for non-advanced systemic mastocytosis (SM). (pharmalive.com)
  • Pediatric mastocytosis is a clonal disease associated with D816V and other activating c-KIT mutations. (medscape.com)
  • Mastocytosis is an unusual clonal disease of the hematopoietic stem cell. (nih.gov)
  • In humans, mastocytosis refers to a heterogeneous group of disorders (most of which have been shown to be clonal and includes the human equivalent of mast cell tumors) characterized by abnormal growth and accumulation of mast cells in one or more organ systems. (nih.gov)
  • People affected by mastocytosis are susceptible to a variety of symptoms, including itching, hives, and anaphylactic shock, caused by the release of histamine and other pro-inflammatory substances from mast cells. (wikipedia.org)
  • H2 antihistamines, such as ranitidine, cimetidine, or famotidine, are used to treat gastric hypersecretion and peptic ulcer disease associated with histamine release in mastocytosis (Worobec, 2000). (medscape.com)
  • Mastocytosis and adverse reactions to biogenic amines and histamine-releasing foods: what is the evidence? (medscape.com)
  • 5. Immunohistochemical properties of bone marrow mast cells in systemic mastocytosis: evidence for expression of CD2, CD117/Kit, and bcl-x(L). (nih.gov)
  • Mastocytosis, a type of mast cell disease, is a rare disorder affecting both children and adults caused by the accumulation of functionally defective mast cells (also called mastocytes) and CD34+ mast cell precursors. (wikipedia.org)
  • Systemic mastocytosis is a blood disorder that can affect many different body systems. (medlineplus.gov)
  • Mastocytosis is a rare and underdiagnosed disorder characterized by mast cell proliferation in the tissues and organs of the body. (nih.gov)
  • Mastocytosis is a relatively infrequent disorder characterized by mast cell proliferation within primarily the skin, but also various organs such as the bone marrow, liver, spleen, lymph nodes and the gastrointestinal system. (aai.org.tr)
  • Mastocytosis is a rare disorder characterized by pathologic mast cell degranulation. (bvsalud.org)
  • Dev PP, Lakhani R, Bansal S, Khunger N. Bullous mastocytosis: A rare but challenging diagnosis in infancy. (cosmoderma.org)
  • Histopathology confirmed the diagnosis of bullous mastocytosis by showing a hyperkeratotic epidermis with acanthosis and focal subepidermal vesiculation with sheets of mast cells arranged in dermis [ Figure 2a ]. (cosmoderma.org)
  • 9. Bullous mastocytosis: report of a patient and a brief review of the literature. (nih.gov)
  • Cite this: Unmasking Mastocytosis - Medscape - Feb 01, 2003. (medscape.com)
  • These modifications potentially allow wider use of the assay to explore the role of PGD2 in human disease and in the routine biochemical diagnosis of systemic mastocytosis and other disorders of mast cell activation. (nih.gov)
  • Mastocytosis represents a group of disparate disorders of mast cell proliferation. (clinicaladvisor.com)
  • In systemic mastocytosis, KIT gene mutations are somatic, which means they are acquired during a person's lifetime. (medlineplus.gov)
  • Because mast cells play a role in allergic reactions, the symptoms of mastocytosis often are similar to the symptoms of an allergic reaction. (wikipedia.org)
  • There are five subtypes of systemic mastocytosis, which are differentiated by their severity and the signs and symptoms. (medlineplus.gov)
  • Mastocytosis is a rare disease characterized by an overabundance of mast cells in one or more organs. (cosmoderma.org)
  • In children, mastocytosis most commonly affects the skin however for young people like Tayla with the much rarer systemic mastocytosis, mast cells gather in different parts of the body, affecting internal organs, bone marrow and digestive tract. (rch.org.au)
  • Because some of the manifestations of mastocytosis are nonspecific and can be mimicked by other diseases, there is a risk of two types of diagnostic error: Mastocytosis may remain undiagnosed when it is actually present, or it may be diagnosed even though morphological and molecular findings rule out mastocytosis. (nih.gov)
  • Symptomatic manifestations of systemic mastocytosis can either be triggered spontaneously or be precipitated by a variety of situations, stimuli, and exposures. (nih.gov)
  • Gastrointestinal manifestations of systemic mastocytosis. (nih.gov)
  • Gastrointestinal involvement and manifestations in systemic mastocytosis. (nih.gov)
  • Due to an extensive accumulation of AML blast cells that obscured the mast cell infiltrates, mastocytosis was not evident at the first diagnosis. (banglajol.info)
  • And when you compare the symptom burden and systemic mastocytosis to other diagnoses, we see that this is looking at SF 12 symptom scores, it is actually very comparable to other disorders that you see here, like colorectal cancer, anemia, depression, and so on. (reachmd.com)
  • Resistance to imatinib has been recurrently reported in systemic mastocytosis (SM) carrying exon 17 KIT mutations. (qxmd.com)
  • Mast cell leukemia is the rarest and most severe type of systemic mastocytosis. (medlineplus.gov)
  • Mastocytosis is an uncommon abnormal accumulation of mast cells in the skin and sometimes in various other parts of the body. (msdmanuals.com)