Towards the pharmacotherapy of hairy cell leukaemia

Hairy cell leukaemia (HCL) offers one of the few examples of rapid progress in the development of effective treatments for chronic lymphoproliferative disorders. After the first description of HCL as a separate disease in 1958, splenectomy was the treatment of choice, but rarely resulted in remission of disease and had scarce benefit on survival. In 1984, IFN-alpha became the first agent able to significantly modify the prognosis of HCL by inducing high response rates and long-term remissions. More recently, purine analogues have significantly further increased the percentages of remissions, with a reduced risk of relapse and are now generally used as first-line treatment. Monoclonal antibodies targeting CD20, CD22 and CD25 antigens, have also shown responses for resistant or relapsing disease. This article will review the current treatment strategies for HCL.