Mast cell leukemia with ascites and multiple organ damage,case report

Introduction: Mast cell leukemia, a rare subtype of systemic Mastocytosis is defined by bone marrow involvement with atypical mast cells, more than 20% of bone marrow aspirate, and named aleukemic if less than 10% of WBC are mast cells. Case Presentation: We met a case of aleukemic variant presenting with anemia and ascites, for more than 2 years, referred for bone marrow evaluation, suspicious of leukemia. Our findings included aggregates of oval and spindle shaped hypogranulated atypical mast cells, on bone marrow aspiration and biopsy, without mast cell distribution in peripheral blood smear. Further assessments showed positive reaction of mast cells metachromatic granules with Giemsa and Toluidine blue stains, weakly expression of CD117 and CD45 by immunohistochemistery stain, and marked elevated level of serum Tryptase. Clinical and radiologic investigations revealed generalized lymphadenopathy, and massive hepatosplenomegaly, followed by cervical lymphadenectomy, and liver wedge biopsy. During operation, suspicious peritoneal lesions had been identified, underwent excisional biopsy. Microscopic evaluations showed both lymph node and liver involvement by cancer cells with features of peritoneal seeding. Conclusion: MCL is a very rare form of Aggressive systemic Mastocytosis, characterized by the leukemic proliferation of MCs, with frequent multiple organ involvement such as the liver, peritoneum, spleen, and bone marrow. MCL diagnosis requires features of involvement of bone marrow (BM) and/or blood by at least 20% atypical mast cells as well as the neoplastic mast cell infiltration of extracutaneousorgans.

کلیدواژه ها:

mast cell ، mastocytosis ، aleukemic variant of mast cell leukemia