Systemic mastocytosis.

Abstract

A 64-year-old man was admitted to the Hematology ward in July 2012 due to neutropenia and thrombocytopenia. The only symptom in the month preceding his admission was significant weight loss. Based on clinical and laboratory investigations, specifically bone marrow aspiration and biopsy (Figure), the patient was diagnosed with aggressive systemic mastocytosis (WHO 2008 ICD-0 code 9741/3). The patient initially received treatment with cladribine 8.5 mg once daily in 2-hour intravenous infusion for a 5-day period/28-day cycle. Following the completion of a 5-cycle treatment course with cladribine, thrombocytopenia improved, but there was no significant change in neutropenia, while the hematocrit remained stable. However, the patient did not show any clinical improvement. Thus, he was reevaluated with bone marrow biopsy, which showed 40% infiltration by malignant mast cells. There was underlying disease of myeloblastic/myeloproliferative neoplasm unclassifiable with 3% IMAGES IN MEDICINE