Systemic mastocytosis

Discussion:

Skeletal abnormalities include, in decreasing frequency; osteopenia/osteoporosis, osteosclerosis, or a combination of both. Osteolytic lesions can be well or poorly demarcated and may be surrounded by a sclerotic zone. 

Common abdominal imaging findings associated with systemic mastocytosis are hepatosplenomegaly, retroperitoneal adenopathy, periportal adenopathy, mesenteric adenopathy, thickening of the omentum and the mesentery as well as ascites.

Less common abdominal findings included hepatofugal portal venous flow, Budd-Chiari syndrome, cavernous transformation of the portal vein, ovarian mass, and complications such as chloroma.

This patient diagnosis was confirmed by a skin biopsy showing a skin fragment with dense dermal collagen bundles, with papillary dark nuclei & scanty cytoplasm. Cells gave positive reaction to CD 117 and toluidine blue. Giemsa was negative. Picture consistent with mastocytosis.

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