2015 ESMO指南：毛细胞白血病的诊断、治疗及随访

Classical hairy cell leukaemia (HCL) is a B-cell chronic lymphoproliferative disorder characterised by splenomegaly, pancytopaenia and bone marrow involvement with fibrosis. HCL represents 2% of adult leukaemia. Approximately 1600 new cases per year are diagnosed in Europe, with a median age of 52 years at the time of diagnosis. The disease occurs more often in men than in women, with a sex ratio of approximately 4:1. In the USA, a higher frequency of HCL is observed among white Americans than among African-Americans or Asians, as well as in patients following exposure to the herbicide ‘Agent Orange’, used during the Vietnam War. HCL variant (HCL-V) is classified among the unclassifiable splenic B-cell leukaemia/lymphoma that is no longer biologically related to classical HCL. It is included in the World Health Organization (WHO) classification as a provisional entity. HCL-V is an uncommon disorder, accounting for approximately 0.4% of chronic lymphoid malignancies and 10% of all HCL cases, without sexual predominance. The median age of the patients is 71 years.