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“Hairy cell leukemia (HCL) was initially recognized as a distinct clinical

and pathologic entity by Bouroncle and colleagues in 1958 [1]. Initially called leukemic reticuloendotheleosis, this rare chronic leukemia features a distinctive malignant cell characterized by a spongy appearance of the nucleus and a blue cytoplasm with an irregular, serrated border. While the cell of origin of this leukemia has been ascribed to a mature monoclonal B cell based upon the expression of CD19, surface immunoglobulin, and clonal rearrangements of immunoglobulin genes, recent studies suggest that the pathogenesis of this disorder involves mutations in the hematopoietic stem cells [2]. HCL is a rare leukemia, comprising only 2% of all leukemias and approximately 8% of all lymphoproliferative disorders, with an estimated 900 new cases diagnosed each year in the United States according to SEER data. The epidemiology remains only partially elucidated, with selleck chemicals llc occupations involving exposure to diesel fuel, organic solvents, large animal farming, and pesticide and herbicide exposure being implicated in the development of the disease [3]. No effect of ionizing radiation was identified. In the U.S., the development

of HCL in patients with prior military exposure to Agent Orange, an herbicide used during the Vietnam War, is now considered a service related illness according to the Institute of Medicine’s Veterans and Agent Orange: Update 2012 published by The National Academies Press in 2014. The most frequently presented complaints are weakness and fatigue, with infection being a feature

in approximately 17% of the patients [4]. In addition to infectious complications, the clinical course of the disease is principally associated with consequences related to bone marrow failure and organomegaly. Historically, splenomegaly was found in up to 96% of the patients [1], however the frequency of marked splenomegaly may be less common as the diagnosis learn more is now being made earlier in the disease course than in the past as a result of abnormalities uncovered on a routine blood count [5] and [6]. The gender distribution of this leukemia remains unexplained, with a 4:1 ratio of men to women. While patients may present at any age throughout adult life, the median age at diagnosis is approximately 55 years old. At the time that this disease was first described, the clinical course was typically associated with a fatal outcome and an estimated median survival of approximately six years, with substantial variability [4]. Mortality was mostly attributable to infection or bleeding complications. Enormous progress has been made over the past two decades, and the majority of patients with classic hairy cell leukemia may now expect to live a near normal life span [7] and [8].