Generalized Mastocytosis

Kammen, E. van

doi:10.1159/000208232

Cited by 6 publications

References 17 publications

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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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Liver findings in generalized mastocytosis. A clinicopathologic study

Horny

Kaiserling

Campbell

et al. 1989

Cancer

105

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Spleen findings in generalized mastocytosis. A clinicopathologic study

Horny

Ruck

Kaiserling

1992

Cancer

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Background. Little information regarding the morphologic findings of the spleen in generalized mastocytosis (GM) is available and no comprehensive review of the literature on this subject has been published. Methods. The authors reviewed their records to study the macroscopic and microscopic features of the spleen in 53 patients; the authors also studied these features in 135 patients reported in the literature. Thus, a total of 188 patients with GM were studied. Results. Splenomegaly was noted in 72% of the patients, including 71% of the patients from the authors' files and 73% of the patients reported in the literature. The spleen weight, recorded in 39 of the patients, ranged from 160 g to 2300 g; in 29 (80%) patients the spleen weighed more than 500 g and in 4 (10%) patients, more than 2000 g. However, mast cell (MC) infiltration of the spleen was recorded in only 65 (34%) patients (patients from authors' records, 39%; patients reported in literature, 32%). The following combinations were extremely rare: splenomegaly without evidence of MC infiltration (n = 1); and histologic evidence of MC infiltration in the absence of splenomegaly (n = 2). The histologic findings of the spleen could be assessed in nine of the patients from the authors' files, eight of whom had MC infiltration. In four patients, infiltration was diffuse and confined mainly to the red pulp; in the other four patients, it was more focal and involved mainly the white pulp. Spleen plasmacytosis was found in eight patients, eosinophilia in five, hemosiderosis in seven, and fibrosis in six. Concurrent acute nonlymphoblastic leukemia was diagnosed in four patients, none of whom had the skin lesions of urticaria pigmentosa. Conclusions. The authors' findings show that (1) splenomegaly is a common finding in GM and often is pronounced; (2) MC infiltration probably is the most important factor in the pathogenesis of splenomegaly in this disease; and (3) two different patterns of MC infiltration (diffuse and focal) can be identified in GM. Cancer 1992; 70:459–468.

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The Mast Cell: Morphology and Ontogeny

Leder¹

1981

New Trends in Allergy

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Generalized Mastocytosis

Cited by 6 publications

References 17 publications

Liver findings in generalized mastocytosis. A clinicopathologic study

Liver findings in generalized mastocytosis. A clinicopathologic study

Spleen findings in generalized mastocytosis. A clinicopathologic study

The Mast Cell: Morphology and Ontogeny

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