Context: Hairy cell leukemia HCL is a rare, low grade, B-cell neoplasm with a characteristic morphologic and immunophenotypic profile. It has to be distinguished from chronic lymphoproliferative disorders because of different treatment protocol and clinical course. Aims: To evaluate clinicopathological features including immunophenotypic analysis of cases diagnosed as HCL. Materials and Methods: The present study included 28 cases diagnosed over a period of nine years Clinical presentation, complete blood count, bone marrow aspirate, and flow cytometric analysis of cases were reviewed.
Hairy cell leukemia
Hairy Cell Leukemia: Rare and Highly Treatable | Everyday Health
Hairy cell leukemia HCL is a neoplasm of mature B lymphoid cells. It affects predominately middle-aged to elderly adults, with a median age of 50 years. It has been diagnosed in younger patients, but is exceptionally uncommon in children. It has a male predominance, with a male to female ratio of approximately 5 to 1. Most common clinical symptoms include weakness and fatigue, as well as left upper quadrant pain.
Hairy Cell Leukemia Treatment (PDQ®)–Patient Version
Hairy cell leukemia HCL is a rare, low-grade mature B-cell neoplasm with a characteristic clinical, morphological, immunophenotypic, and more recently described molecular BRAF p. VE mutation profile. It typically affects middle-aged to elderly male who present with pancytopenia and massive splenomegaly. Lymphadenopathy is usually not seen.
The standard first-line treatment, which has remained unchanged for the past 25 to 30 years, is single-agent therapy with a purine analogue, either cladribine or pentostatin. This approach produces a high rate of complete remission. Residual traces of HCL cells, referred to as minimal residual disease, are present in most patients and cause frequent relapse. Repeated treatment with a purine analogue can restore remission, but at decreasing rates and with increasing cumulative toxicity.