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Leukemia & Lymphoma, June 2011; 52(S2): 21–24


Long-term results for pentostatin and cladribine treatment of hairy cell leukemia


CLAIRE E. DEARDEN, MONICA ELSE, & DANIEL CATOVSKY The Royal Marsden NHS Foundation Trust and The Institute of Cancer Research, UK


Abstract Over the past 25 years we have collected data at our institution from 242 patients with hairy cell leukemia (HCL), treated


with pentostatin (n¼188) or cladribine (n¼54), with a median follow-up of 16 years. From this we have been able to conclude that there is no significant difference in outcome between the two agents either at first or subsequent lines of therapy. Overall, the complete response (CR) rate is 81% and the median disease-free survival (DFS) is 16 years. After relapse or non-response patients can be successfully retreated with pentostatin or cladribine achieving a lower rate of CRs with each line of therapy, although these remain equally durable. Complete response and pretreatment counts of hemoglobin410 g/dL together with platelets41006109/L are associated with the longest DFS. Importantly, for patients achieving a CR the DFS is five times as long as for those achieving a partial response (PR). Patients still in CR at 5 years have only a 25% risk of relapse by 15 years. Outcomes for patients with recurrent disease have improved with the addition of rituximab to either purine analog. Overall, only eight patients have died of HCL-related causes. Patients with HCL who achieve a CR can expect a normal lifespan.


Keywords: Hairy cell leukemia, pentostatin, cladribine Introduction


In the 52 years since hairy cell leukemia (HCL) was first described by Bouroncle et al. [1], there have been major advances in the management of this rare disease. Thirty years ago the median survival for patients with HCL was only 4 years [2]. The introduction of the purine analogs, pentostatin and cladribine, in the 1980s has transformed this prog- nosis. Studies have consistently reported overall response rates of more than 85% with median disease-free survival (DFS) of 15–16 years [3–6]. We have recently published outcome data for 233 patients with HCL treated with either pentostatin or cladribine [7]. The median survival in our series of patients achieving a complete response (CR) was not yet reached at 20 years. This review focuses on the implications of these results for clinical practice.


Pentostatin and cladribine are equally effective treatments for hairy cell leukemia


The Royal Marsden Hospital (RMH)/Institute of Cancer Research clinical database now includes 251


patients with HCL who have received treatment with a single-agent purine analog between 1986 and 2010. Of these, 242 cases were available for analysis. The median age at diagnosis was 49 (range 23–76) years with a male:female ratio of 4:1. All cases of HCL- variant were excluded from this series following careful review. The statistical methods used were as previously described [7]. Pentostatin was given as first-line therapy to 188


patients and cladribine to 54. The median follow-up was 16.0 years from diagnosis. Most patients who did not respond to initial therapy received second-line treatment with the alternative purine analog. At recurrence, the majority of patients were retreated with either the same or the alternative drug. From 2003, the combination of the monoclonal antibody rituximab with pentostatin or cladribine has been given to selected patients with recurrent disease [8] (also see the article by Else et al. in this Supplement). Standard treatment protocols for both agents were


followed [7]. More recently we have switched from a 7-day continuous intravenous (IV) infusion regimen of cladribine 0.1 mg/kg/day to subcutaneous cladri- bine at a dose of 0.14 mg/kg daily for 5 days. This has


Correspondence: Claire Dearden, Department of Haemato-Oncology, The Royal Marsden NHS Foundation Trust, Downs Road, Sutton, Surrey SM2 5PT, UK. Tel: 0208-661-3655. Fax: 0208-642-6782. E-mail: Claire.dearden@rmh.nhs.uk


ISSN 1042-8194 print/ISSN 1029-2403 online  2011 Informa UK, Ltd. DOI: 10.3109/10428194.2011.565093


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