Hairy cell leukemia

Hairy cell leukemia is a chronic lymphoproliferative disorder that has been recognized as a separate clinical pathologic entity for the last 25 years. After a decade of discussions about the origin of the neoplastic cell, it has now been well established that hairy cells represent a certain, rather mature stage of B-cell differentiation. Evidence for this has been derived from studies using immunophenotyping with monoclonal and polyclonal antibodies, cytochemistry, and immunoglobulin gene rearrangement. For many years, splenectomy was the only therapy of proven value in hairy cell leukemia. For patients who showed insufficient response to the operation, chemotherapy with low-dose alkylating agents was moderately successful, whereas polychemotherapy often resulted in excessive toxicity. More recently, therapy with alpha-interferon has been shown to be very promising, whereas deoxycoformicin may be an attractive alternative. These new advances in immunology and therapy are reviewed.