فهرست نشریات علمی دسترسی آزاد ایران

The key purpose of this report is to present an uncommon case of hairy cell leukemia variant (HCLv). HCLv is found to be a striking clinico-pathological condition which encompasses the features of classical hairy cell leukemia (cHCL) as well as B cell prolymphocytic leukemia. It is a rather rare entity accounting for 0.4% of all chronic lymphoid malignancies and about 10% of all HCL cases diagnosed worldwide. A 57-year-old man presented with pain abdomen, loss of weight, spikes of fever and massive splenomegaly to our center. Complete blood count showed leucocytosis with 60% blasts on smear. Bone marrow studies were suggestive of acute leukemia like picture. Flow cytometry revealed the markers CD11c, CD19 and CD103 to be positive. This confirmed the diagnosis of HCLv. Other differential diagnoses such as splenic marginal zone lymphoma, B cell Prolymphocytic Leukemia and cHCL should be ruled out before confirming HCLv as each of them have different responses to antimetabolites, splenectomy and BRAF inhibitors.