Abstract
Hairy cell leukemia is relatively rare, chronic low grade B-cell leukemia. It is characterized morphologically by cells with abundant cytoplasm and “hair”-like projections that can be found within the peripheral blood, bone marrow or splenic pulp. Like many cancers, the treatment for hairy cell leukemia has evolved considerably in the last several years. In the mid 1980s, the acceptable treatment was splenectomy and later interferon therapy, which is quickly falling out of favor with the advent and acceptance of purine analog therapy. In addition, we now have several biologic agents that are coming into favor based on their improved success and favorable toxicity. In addition, several studies are currently looking into the efficacy of purine analogs in conjunction with biologic agents. Rituximab, alemtuzumab and recombinant immunotoxins are being studied with limited success in hairy cell leukemia. The use of kinase inhibition is also being studied at this time. Advances in understanding the biology of hairy cell leukemia could potentially increase more treatment options.
Highlights
Hairy cell leukemia (HCL) is a chronic, low grade, non-Hodgkin lymphoma. This disease is relatively rare and represents 2% of all lymphomas [1]. It is characterized by the accumulation of small, mature B-cell lymphoid cells with abundant cytoplasm and "hairy" projections that are found within the peripheral blood, bone marrow, and splenic red pulp [1,2]
Significant synergy was noted, when rituximab was combined with Purine Analogues (PA) in relapsed and refractory HCL patients
LMB-2 is an anti-CD25 recombinant immunotoxin, and its role in treating HCL in chemotherapy resistant patients is under evaluation with randomized clinical trials
Summary
Hairy cell leukemia (HCL) is a chronic, low grade, non-Hodgkin lymphoma This disease is relatively rare and represents 2% of all lymphomas [1]. It is characterized by the accumulation of small, mature B-cell lymphoid cells with abundant cytoplasm and "hairy" projections that are found within the peripheral blood, bone marrow, and splenic red pulp [1,2]. Most patients show excellent long-term response to treatment with a single-agent purine analog. The increasing number of patients, who either relapse or become refractory to purine analog therapy, led to more studies for a better understanding of the HCL pathobiology and more effective treatment options. Complete response rates were similar between patients treated with cladribine (84%) or pentostatin (82%). Patients with refractory or relapse disease usually received a further course of either drug
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