2-CdA in the treatment of hairy cell leukemia: a review of long-term follow-up.

Hairy cell leukemia is a rare, indolent, chronic lymphoproliferative disorder characterized by the proliferation of a malignant B-cell clone with irregular cytoplasmic projections. Treatment with purine analogs such as 2-chlorodeoxyadenosine (2-CdA) is associated with excellent remission rates and long-term survival. Although the majority of patients achieve a complete remission (CR), most have minimal residual disease detected by sensitive methods. Despite the long term disease-free survival, approximately 36% of patients relapse and many require further therapy. Repeat administration of 2-CdA is very effective in achieving a second CR. Recently, new agents such rituximab and BL22 were shown to be effective in the treatment of relapsed and refractory disease.