Current treatment options in hairy cell leukemia and hairy cell leukemia variant

Hairy cell leukemia (HCL) is a chronic B-cell lymphoproliferative disorder characterized by splenomegaly, pancytopenia and circulating lymphocytes displaying prominent cytoplasmic projections. HCL has usually an indolent course and the patients with asymptomatic disease do not require therapy. Treatment of progressive symptomatic HCL includes a variety of pharmacological approaches such as interferon-alpha (IFN-alpha), pentostatin (DCF) and cladribine (2-CdA), which have significantly improved the disease prognosis. 2-CdA and DCF seem to induce a similar high response rate and a long overall survival. They are also active in relapsed patients. More recently high activity of anti-CD20 monoclonal antibody (rituximab) and anti-CD25 (LMB-2) and anti-CD22 (BL-22) immunotoxins have increased the number of therapeutic options for HCL. Splenectomy may be still indicated in patients with massive, symptomatic splenomegaly or results in severe cytopenia. IFN-alpha may have a place in patients with very severe cytopenia, in HCL in pregnancy and in patients who have failed prior therapy with purine nucleoside analogs. HCL variant (HCL-V) is a distinct clinico-pathological entity which seems to be resistant to IFN-alpha and purine nucleoside analogs - DCF and 2-CdA. However, preliminary observations suggest that monoclonal antibodies - rituximab and BL-22 immunotoxin are highly active in this disorder even refractory to 2-CdA. In this review current therapeutic strategies in HCL and HCL-V are presented.