Treating hairy cell leukemia

Hairy cell leukemia (HCL) tends to be slow growing. Patients without symptoms often don't need to be treated right away, but they do need to have careful follow-up exams. These are done every few months to check for disease progression and appearance of symptoms. Some patients with HCL live for many years without having any symptoms or receiving any treatment.

Treatment may be advised for HCL patients with low blood cell counts, recurrent infections, or an enlarged spleen or lymph nodes. Treatment is most often with one of the purine analog drugs -- either cladribine (2-CdA) or pentostatin. Up to 80% to 90% of patients respond to these drugs, and the responses last more than 5 years in most patients. Even if HCL recurs, many cases will respond to a second treatment with these drugs.

Giving rituximab after these drugs may get rid of any remaining disease in people who haven't fully responded. Because this is a fairly rare disease, too few people have been treated with rituximab to know if it will make a long-term difference.

In rare cases, HCL may not respond to chemotherapy. Rituximab or interferon-alfa, a type of biologic therapy, may be helpful. If a patient is uncomfortable because of an enlarged spleen, removing the spleen by surgery (splenectomy) can often help relieve pain or other symptoms.