Gastrointestinal involvement in systemic mastocytosis.

Ammann, R; Vetter, D; Deyhle, P; Tschen, H H; Sulser, H; M, Schmid

doi:10.1136/gut.17.2.107

Cited by 72 publications

(22 citation statements)

References 38 publications

(42 reference statements)

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“…Such nodularity has been documented by others, both radiologically and endoscopically. 2,[6][7][8]18,19,23,[34][35][36][37][38] Other nonspecific findings seen in this series and reported in the literature include erosions, loss of mucosal folds and friability. A substantial proportion of cases shows no obvious endoscopic or radiological abnormality.…”

Section: Discussionmentioning

confidence: 96%

“…These findings are similar to those reported in other small series and case reports. 2,[5][6][7][8][9][10][11]13,14,[16][17][18][21][22][23]26,30,31 Malabsorption, which occurred in two patients in our series is well described in systemic mastocytosis, 2,5,7,9,10,16,23,30,31 occurring in 5-30% of patients. 30,32 While all patients in our series were women, the literature indicates an equal sex distribution for systemic mastocytosis in general 33 and for those cases with direct gastrointestinal involvement.…”

Section: Discussionmentioning

confidence: 97%

“…30,32 While all patients in our series were women, the literature indicates an equal sex distribution for systemic mastocytosis in general 33 and for those cases with direct gastrointestinal involvement. [5][6][7][8][9][10][11]13,[16][17][18][19][21][22][23]31 Endoscopically and grossly, the most frequent and distinctive finding in our series was that of mucosal nodularity (present in four of five patients). Such nodularity has been documented by others, both radiologically and endoscopically.…”

Section: Discussionmentioning

confidence: 99%

“…Several studies have reported an increase in lamina propria mast cells, 7,8,[11][12][13][14]16,22 while others have not. 3,4,6,9,15,20 Such variation may reflect genuine differences among patients but may also be the result of differences in selection criteria, sampling variability and stains used to highlight mast cells. Many early studies used histochemical stains such as toluidine blue and Giemsa; these stains require the presence of intracytoplasmic mast cell granules and likely underestimate mast cell numbers compared to immunohistochemical stains such as mast cell tryptase and CD117, employed in more recent studies.…”

Section: Discussionmentioning

confidence: 99%

“…Indeed, three of five patients in our series had colonoscopic findings considered to be consistent with inflammatory bowel disease (although one had coexistent ulcerative colitis). The issue may be further complicated by the fact that the lamina propria infiltrate in systemic mastocytosis may include variable numbers of lymphocytes and plasma cells, [6][7][8]10,14,23,31,39 and that inflammatory bowel disease may show a prominence of mast cells or eosinophils. [40][41][42] A recent report of systemic mastocytosis mimicking Crohn's disease highlights the potential for misdiagnosis of these conditions.…”

Section: Discussionmentioning

confidence: 99%

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Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

et al. 2008

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Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

et al. 2008

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Urticaria Pigmentosa

et al. 1991

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Nine patients with adult-onset urticaria pigmentosa were studied for the incidence of extracutaneous mast cell involvement and the efficacy of potent topical corticosteroid therapy for cutaneous lesions. Seven of the nine patients had increased mast cells in the marrow biopsy specimens, and five patients had focal aggregates of mast cells. The bone scan was abnormal in one patient. Liver-spleen scans revealed a shift of colloid uptake from liver to spleen in four patients. No abnormal gastrointestinal tract roentgenograms were obtained. Urinary histamine metabolites correlated with nodular bone marrow involvement, but not with other parameters. Results of the psychoneurologic testing revealed significant deviation from the norm with a verbal memory deficit in all nine patients and abnormalities on the Minnesota Multiphasic Personality Inventory in four patients. All nine patients were treated with 0.05% betamethasone dipropionate ointment under occlusion over half of the body nightly for 6 weeks. Seven of nine patients treated responded with almost complete resolution of their lesions. Hypothalamic pituitary adrenal axis suppression was evaluated with intramuscular cosyntropin stimulation and metyrapone administration during treatment. Only two patients, both of whom used the medication improperly, developed transient abnormalities. Slow return of lesions was noted 6 months after completion of therapy. Remissions could be lengthened with single weekly applications of topical steroids. Systemic involvement is frequent in patients with cutaneous mast cell disease and it is best demonstrated by bone marrow biopsy. Mast cell lesions can be safely and effectively treated with topical steroids in motivated patients.

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Liver findings in generalized mastocytosis. A clinicopathologic study

Horny

Kaiserling

Campbell

et al. 1989

Cancer

105

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Although the liver is one of the four organs most often involved in generalized mastocytosis (GM), little is known about macroscopic and microscopic liver findings in this rare disease. This study included 182 patients with GM (confirmed in most by bone marrow histologic study), comprising 52 cases of our own and 130 reported in the literature. Hepatomegaly was found in 131 (72%) of the 182 patients, cirrhosis in seven (4%), and periportal fibrosis in 25 (14%). Mast cell (MC) infiltration of the liver was confirmed histologically in 77 (42%). Liver specimens were available for further histologic investigation in 11 of our own cases of GM. Nine of these contained MC aggregates. Mast cells were found predominantly in the portal tracts but numerous MC also were loosely scattered throughout the sinusoids. Diagnostic confusion of GM with reactive lesions of the liver is unlikely to occur since MC, according to our own observations and the available literature, are found only in very low numbers in normal liver tissue, where they occur mainly in the portal tracts. Reliable identification of MC does, however, require special stains, like Giemsa, toluidine blue, or naphthol AS-D chloroacetate esterase.

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Gastrointestinal involvement in systemic mastocytosis.

Cited by 72 publications

References 38 publications

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

Systemic mastocytosis involving the gastrointestinal tract: clinicopathologic and molecular study of five cases

Urticaria Pigmentosa

Liver findings in generalized mastocytosis. A clinicopathologic study

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