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Aggressive systemic mastocytosis complicated by protein-losing enteropathy
U Mickys1, A Barakauskiene, C De Wolf-Peeters
1National Center of Pathology, P. Baublio 5, LT-08406 Vilnius, Lithuania.
Summary
Aggressive systemic mastocytosis can affect the gastrointestinal tract, causing severe symptoms like diarrhea and vomiting. This case highlights a rare association with protein-losing enteropathy, successfully treated with prednisolone and interferon-alpha.
Area of Science:
- Gastroenterology
- Hematology
- Oncology
Background:
- Systemic mastocytosis is a rare disorder characterized by abnormal mast cell proliferation.
- Gastrointestinal involvement is common but protein-losing enteropathy is an unusual complication.
Observation:
- A 46-year-old woman presented with chronic diarrhea, vomiting, malnutrition, and edema.
- Endoscopy revealed duodenal erosions and colonic pseudopolypoid lesions.
- Biopsies showed atypical mast cells in the lamina propria and bone marrow.
Findings:
- Immunophenotyping confirmed mast cells (CD68+, c-kit/CD117+, tryptase+).
- Bone marrow biopsy revealed diffuse infiltration by atypical mast cells, consistent with aggressive systemic mastocytosis.
- The patient had protein-losing enteropathy secondary to gastrointestinal mastocytosis.
Implications:
- This case represents a previously unreported association between aggressive systemic mastocytosis and protein-losing enteropathy.
- Early diagnosis and appropriate treatment are crucial for managing this rare condition.
- Further research may elucidate the mechanisms linking mastocytosis to protein-losing enteropathy.
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