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We treated 11 patients (nine men and two women) with hairy cell leukemia with low doses of pentostatin (2'-deoxycoformycin). As of January 1987, 10 patients (91%) were in complete remission (CR) and one (9%) was in partial remission. Thus, the overall response rate was 100%. Maintenance therapy was not given once CR was attained, but no patient in CR relapsed: remission durations were from 40+ to 9+ months. For hairy cell leukemia, pentostatin is a better treatment than splenectomy and probably is superior to interferon, but further studies are needed to better define its role in this disease.