Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Systemic mastocytosis occurred as a fatal event in a patient with long-standing polycythemia vera. The patient had been treated over the course of 21 yr with radioactive phosphorus. Possible relationships between mastocytosis and polycythemia vera, and also between mastocytosis and treatment with ionizing radiation, are discussed. Histopathologic and electron microscopic findings are illustrated. Difficulties in establishing the diagnosis of mast cell disease in this setting are also described.
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PMID:Systemic mastocytosis in a patient with polycythemia vera treated with radioactive phosphorus. 30 Feb 55

Malignant Systemic Mastocytosis is a very rare condition. Only about less than 40 well documented cases have been reported as per the available literature. The paper presents the case report of a 54 year old male patient who presented with huge hepatosplenomegaly and abdominal lymphadenopathy. Splenectomy specimen was 17 x 16 x 10 cm size with cut surface studded with numerous tiny 1-2 mm nodules. Histologic sections of spleen showed extensive mast cell (typical and atypical) infiltrates. Liver biopsy and abdominal lymphnode biopsy specimens and bone marrow smears also showed similar infiltration by mast cells. Special stains done for non-specific esterase and chloracetate esterase showed strong positivity for mast cells. The results of immunohistochemical and electron microscopic studies are also presented.
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PMID:Malignant systemic mastocytosis. 129 4

Systemic mastocytosis is a rare disease of mast cell proliferation with cutaneous and multi-visceral involvement. Portal hypertension and ascites are rare manifestations of systemic mastocytosis. We report a case of systemic mastocytosis presenting with extensive nodular cutaneous lesions and hepatic dysfunction, manifested by portal hypertension (ascites, splenomegaly) and derangement of metabolic function (hyperammonemia, hypoalbuminemia, hypocholesterolemia), a picture resembling that of a common cirrhotic form. The correct diagnosis was established only after tissue sections were appropriately stained for mast cells. On the basis of our and other observations we suggest that systemic mastocytosis be added to the list of infiltrative diseases of the liver with potential evolution to portal hypertension and compromise of biochemical functions.
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PMID:[Systemic mastocytosis with portal hypertension and hepatocellular failure]. 143 8

Systemic mast cell disease is a rare condition, in which the frequent involvement of bones is likely to raise diagnostic problems. There are other concomitant visceral involvement as a rule. The skin lesions, if present and recognized, are suggestive. The positive diagnosis of bone lesions can be ensured by biopsy. On the basis of one clinical case, we specify the clinical and radiological appearance of systemic mast cell disease.
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PMID:[Bone involvement with osteolytic predominance in systemic mastocytosis. A clinical case]. 147 20

Systemic mastocytosis is a rare condition in which mast cells infiltrate various organs, including the skeleton. Because the mast cell secretes various bioactive substances that may induce bone resorption, this condition may cause generalized osteoporosis. We describe a case of a 28-year-old woman who presented with a painful thoracolumbar kyphosis due to generalized osteopenia and multiple pathological compression fractures and was found to have mastocytosis. She underwent operative stabilization of her kyphotic deformity with anterior interbody fusion and posterior Cotrel-Dubousset (CD) instrumentation and fusion. We conclude that mastocytosis should be suspected in an atypical case of so-called idiopathic osteoporosis.
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PMID:Systemic mastocytosis presenting with severe spinal osteopenia and multiple compression fractures. 180 68

The first known case of systemic mast cell disease associated with a germ cell tumor is reported. Six months after the complete remission of a primary mediastinal germ cell tumor treated by chemotherapy and resection, a young man had a series of episodes of hypotension and syncope and, a few months later, a gastric hemorrhage and coagulation disorder. The diagnosis of systemic mast cell disease was made in view of bone marrow and liver biopsies. A transient circulating heparin-like anticoagulant was noted. The occurrence in a short period of these two unusual conditions, given previous knowledge of hematologic disorders associated with germ cell tumors, suggests that the present association is not a coincidence. Systemic mast cell disease should be considered among the hematologic disorders associated with a germ cell tumor.
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PMID:Systemic mast cell disease associated with primary mediastinal germ cell tumor. 184 96

Systemic mastocytosis is characterized by an abnormal proliferation of tissue mast cells. Symptoms of mastocytosis are primarily attributed to the release of mast cell mediators during episodes of systemic activation of the excessive numbers of mast cells. Thus, biochemical evidence for the release of increased quantities of mast cell secretory products can suggest or confirm, depending on the clinical situation, a diagnosis of systemic mastocytosis. A major advantage of the biochemical approach to the diagnosis of systemic mast cell disease is that it has allowed the recognition of a class of patients in whom episodes of systemic mastocytes activation can be unequivocally documented biochemically but in whom clear-cut evidence of abnormal mast cell proliferation is lacking by current histologic criteria. Although the release of increased quantities of mast cell mediators can be demonstrated during episodes of mast cell activation in such patients, mediator levels are usually normal at quiescent times. By contrast, patients with proliferative mast cell disease (mastocytosis) usually exhibit chronic overproduction of mast cell mediators. Mast cell secretory products that can be measured in an attempt to obtain biochemical evidence of systemic mast cell activation include histamine, prostaglandin D2, tryptase, and heparin. The analytical approaches to assessing release of those individual mast cell products are evaluated. In general, the diagnosis and investigation of patients with systemic mast cell activation can best be accomplished by concerted use of histologic examination of key tissues together with analysis of chemical markers of the mast cell.
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PMID:Biochemical diagnosis of systemic mast cell disorders. 200 47

The gastrointestinal tract is a rich source of mast cells with an enormous surface area that permits a high degree of interaction between the mast cell and intestinal luminal contents. The active metabolic products of the mast cell influence gastrointestinal secretion, absorption, and motility through paracrine effects of local mast cell degranulation and also cause systemic effects through the release of cellular products into the blood stream. Systemic mastocytosis influences physiologic function through the systemic effects of mast cell products released from focal (e.g., bone marrow) or wide spread increases in mast cell number. Local gastrointestinal proliferation of mast cells in response to recognized (e.g., gluten in celiac sprue) or obscure stimuli can alter gastrointestinal function and induce systemic symptoms. Celiac sprue, inflammatory bowel disease, and non-ulcer dyspepsia are three examples of gastrointestinal diseases in which mast cells can be implicated in the pathophysiology of the symptoms.
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PMID:The role of the mast cell in clinical gastrointestinal disease with special reference to systemic mastocytosis. 200 61

Blood findings in 61 cases of generalized mastocytosis (GM) were evaluated. The cases were divided into two major variants: Systemic mastocytosis (SM; n = 34) with urticaria pigmentosa-like skin lesions, and malignant mastocytosis (MM; n = 27), without skin involvement. The following results were obtained: (1) Significant differences between MM and SM were found in the main haematological parameters (erythrocyte, platelet and leucocyte counts and haemoglobin level); normal values were found in 16 of the SM cases, but never in MM. (2) The main pathological findings were: in SM, anaemia (9/34) and leucocytosis (5/34); and in MM, leucocytosis (19/27), monocytosis (14/27), eosinophilia (12/27), bicytopenia (12/27, mostly anaemia with thrombocytopenia), basophilia (10/27) and isolated anaemia (7/27). (3) The major finding was a significant difference between MM and SM in the incidence of myeloproliferative disorders (MPD), myelodysplasia and mast cell leukaemia (MCL): these disorders occurred in 23 (92%) MM patients, but only in two (6%) SM patients (P less than 0.001). The four instances of MCL and two of myelodysplasia all occurred with MM. Of the 19 cases of MPD, six (SM, 1; MM, 5) were acute variants (acute myeloid and myelomonocytic leukaemias) and 13 (SM, 1; MM, 12) were chronic variants. No case of malignant lymphoma was noted. (4) The blood picture in 10 of 13 chronic MPD cases represented an atypical chronic myeloid leukaemia for which the preliminary descriptive term 'mastocytosis-associated MPD' is proposed. (5) A survey of 103 published cases (SM, 77; MM, 26) yielded similar findings, including a high incidence of MPD and MCL in MM. These findings add further weight to the argument for recognizing SM and MM as two separate entities.
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PMID:Blood findings in generalized mastocytosis: evidence of frequent simultaneous occurrence of myeloproliferative disorders. 201 71

Systemic mastocytosis is a rare, often undiagnosed disease characterized by the proliferation of mast cells in several tissues. The clinical symptoms are related to the mast cell infiltrates, but also to the release of numerous mediators. Malignant mastocytosis is a term that refers to two different entities: aggressive mastocytosis, where the outcome with a myeloproliferative disease, where the latter governs the prognosis. The potential severity of these diseases justifies the development of more intensive therapeutic measures.
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PMID:[Systemic mastocytosis and malignant mastocytosis]. 213 89


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