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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mastocytosis is characterized by increased proliferation of mast cells. Two patients had systemic mastocytosis involving the skin and gastrointestinal tract, complicated by malabsorption and tetany. Absorption studies in these patients suggested that the entire small bowel was involved and that the defect was mild in the absence of diarrhea. Small bowel biopsies disclosed infiltration of the lamina propria and submucosa by mast cells, and gastrointestinal tract x-ray films showed nodular densities, edema, and thickening of the bowel wall. Tetany was due in part to combined hypocalcemia, hypomagnesemia, and hypokalemia. Diarrhea and malabsorption were due to mast cell infiltration of the bowel rather than to histamine. patients with signs of systemic mastocytosis should have careful evaluations and be followed up to prevent development of malabsorption and tetany.
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PMID:Tetany, malabsorption, and mastocytosis. 119 Sep 35

Bone mastocytosis is characterized radiographically in some patients by diffuse osteosclerosis and in others by demineralization. The reason for these apparently conflicting bone features is unknown. Bone remodeling and marrow mastocytosis infiltration were studied in nine cases of mastocytosis with bone marrow involvement. Six men, ranging from 42 to 78 years of age, and three women, 43, 55, and 73 years old, comprised the series. Two patients had severe and diffuse osteosclerosis. Seven had diffuse demineralization, with crushed vertebrae in four, suggesting common osteoporosis. In three of the seven, cutaneous mastocytosis was absent. Bone biopsies were undecalcified and stained with toluidine blue. In the seven patients with demineralization, the number of marrow mastocytes was increased (154 +/- 24 versus 2 +/- 0.5/mm2 in normal postmenopausal osteoporosis). Mastocyte nodules covering 1-9% of the marrow area were present in all seven patients. These patients showed a significant increase in remodeling; bone formation rate was increased, coupled with a decrease in mean wall thickness. Concomitantly, osteoclast surfaces were increased, with an increased amount of bone resorbed. The two patients with diffuse osteosclerosis had a markedly different histology; mast cell infiltration was dramatically increased (mastocyte count greater than 1000/mm2) with diffuse marrow fibrosis. Bone volume was increased as well, and most of the bone was woven with an intratrabecular mineralization defect. High bone remodeling and decreased osteoblast activity can explain bone loss in mastocytosis with demineralization. Mastocytosis with osteosclerosis is characterized by a more extensive marrow mast-cell infiltration and fibrosis.
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PMID:Bone mastocytosis. A report of nine cases with a bone histomorphometric study. 160 Jun 67

The onset of mastocytosis occurs between birth and 2 years of age in approximately 55% of all cases; an additional 10% develop the disease before the age of 15 years. Mastocytosis in these age groups differs in many respects from mastocytosis that has its onset in adulthood. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations: either a solitary mastocytoma, urticaria pigmentosa, or, less commonly, diffuse cutaneous mastocytosis. Particularly in infants, bullous eruptions may occur. Mastocytosis in infants and children may involve internal organs, including the bone marrow and the gastrointestinal tract, although such manifestations appear to be less common in children than in adults. Plasma histamine levels may be elevated in pediatric-onset mastocytosis. Treatment usually involves the use of H1 and H2 antihistamines to control itching and to control the hypersecretion of gastric acid that may occur. The prognosis for children with mast cell disease is variable; approximately half of the children with urticaria pigmentosa may experience resolution of lesions and symptoms by adolescence.
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PMID:Pediatric mastocytosis. 170 49

Mastocytosis is a disease characterized by an abnormal increase in mast cells. Manifestations of the disease are provoked in large part by the resultant increase in mast cell-derived mediators, which have a variety of local and systemic effects. Mastocytosis is variable in respect to the organ systems involved, clinical manifestations, and association with hematologic diseases. This has suggested the need for an improved classification scheme to allow assessment of prognosis and therapy. The heterogeneity of the disease patterns in mastocytosis strongly suggests that more than one biologic lesion may occur in the developmental sequence that leads to placement of mature mast cells in tissues.
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PMID:Classification and diagnosis of mastocytosis: current status. 200 48

Mastocytosis comprises a heterogeneous spectrum of clinical manifestations, extending from isolated, benign skin infiltrates to systemic involvement, occasionally with a fatal outcome. After a short survey of the morphology and physiology of the mast cell and the skin lesions of mastocytosis, the involvement of internal organs is reviewed and the differential diagnosis is discussed. The options for therapy are discussed, and the need for continuous monitoring of mastocytosis patients is emphasized.
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PMID:[The clinical spectrum of mastocytosis]. 219 96

Mastocytosis, a disease that varies in its clinical presentation, is usually documented by histologic examination of lesional skin. However, no universally accepted histopathologic criteria exist for establishing the diagnosis of this disease. We have combined the method of morphometric point counting with the mast cell-specific stain, conjugated avidin, to accurately quantify mast cells in cutaneous tissue sections of mastocytosis. Using this histologic approach, we found that macules, papules, and nodules of mast cell disease had from ninefold to nearly a 160-fold greater mast cell content than was observed in normal skin and in several other cutaneous disorders. This technique also permitted the objective histologic stratification of mastocytosis skin lesions according to their mast cell density. Morphometric point counting in conjunction with conjugated avidin offers a simple and accurate method for establishing the diagnosis of mastocytosis.
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PMID:Diagnosis of mastocytosis subsets using a morphometric point counting technique. 244 79

Mastocytosis represents a spectrum of clinical disorders that results from an aberrant proliferation of tissue mast cells. This disease process may be confined to the skin (cutaneous mastocytosis) or may involve multiple organs (systemic mastocytosis). Parameters that are useful in differentiating cutaneous from systemic disorders include patient age, symptom complex, and clinical signs. A wide range of clinical symptoms may be encountered in patients with mastocytosis which result from the release of pharmacologically potent mast cell mediators. Distinct cutaneous patterns resulting from skin mast cell infiltrates can be helpful in identifying patients with systemic involvement. The diagnosis of mastocytosis is confirmed by demonstrating increased tissue mast cells in involved organs. The overall prognosis for patients with proliferative mast cell disease is relatively good, although a small percentage are at risk for developing a fatal neoplastic disorder (malignant mastocytosis). Treatment of mastocytosis is directed at both inhibiting mast cell degranulation and blocking the potential systemic effects of released secretory products. Future therapeutic advances depend upon an improved understanding of the basic mechanisms involved in mast cell mediator release and the forces that govern mast cell growth and development.
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PMID:The spectrum of mastocytosis. 257 53

Mastocytosis is a disease characterized by an increase in the number of tissue mast cells and a concomitant increase in mast cell-derived mediators. To demonstrate the spectrum of skin disease in mastocytosis in the pediatric population, five children with mastocytosis and complaints of urticaria (4/5), bullae/vesicles (3/5), abdominal pain (3/5), flushing (2/5), headache (1/5), and bone pain (1/5) are reviewed. Confirmation of the diagnosis of cutaneous mastocytosis was obtained by histologic examination of a biopsy of lesional skin; however, mast cell numbers in lesional skin did not correlate with plasma histamine levels or the extent of cutaneous involvement. Mastocytosis is a diagnosis that must be recognized in the differential diagnosis of pediatric urticarial diseases.
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PMID:Mastocytosis in infants and children: recognition of patterns of skin disease. 292 86

Systemic mastocytosis, with its diffuse infiltration of mast cells into various organs, has resulted in intestinal malabsorption and bleeding diatheses. The pathophysiology underlying these phenomena is unclear, but may be related to the release of histamine and heparin containing mast cell granules. A patient with systemic mastocytosis had malabsorption and developed massive bleeding after percutaneous liver biopsy. Histologic involvement of skin, duodenum, rectum, liver, and bone marrow was documented. Mastocytosis should be considered in the differential diagnosis of malabsorption.
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PMID:Bleeding after liver biopsy in a patient with systemic mastocytosis and malabsorption. 404 81

Mastocytosis gives rise to clinical symptoms such as flushing, itching and diarrhoea. We report a patient with urticaria pigmentosa without evidence of systemic involvement but with recurrent episodes of diarrhoea. The patient had elevated circulating levels of calcitonin, which might have been a mediator of her diarrhoea. We suggest that serum calcitonin level should be checked in patients with mast cell disease and diarrhoea.
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PMID:Hypercalcitoninaemia in a patient with urticaria pigmentosa. A possible cause of diarrhoea. 673 Oct 41


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