Long-term follow-up of 233 patients with hairy cell leukaemia, treated initially with pentostatin or cladribine, at a median of 16 years from diagnosis |
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Authors: | Monica Else Claire E. Dearden Estella Matutes Juan Garcia-Talavera Ama Z. S. Rohatiner Steve A. N. Johnson Nigel T. J. O'Connor y Haynes Nnenna Osuji Francesco Forconi Francesco Lauria Daniel Catovsky |
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Affiliation: | The Institute of Cancer Research and the Royal Marsden NHS Trust, Sutton, UK;, Hospital Ntra Sra de la Candelaria, Santa Cruz de Tenerife, Spain;, St Bartholomew's Hospital, London;, Taunton and Somerset Hospital, Taunton;, Royal Shrewsbury Hospital, Shrewsbury;, Nottingham City Hospital, Nottingham, UK;, and Ematologia e Trapianti, Universitàdegli studi di Siena - AOUS, Siena, Italy |
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Abstract: | Hairy cell leukaemia (HCL) was first described 50 years ago. Median survival was then 4 years. The purine analogues, introduced in the 1980s, transformed this prognosis. We reviewed data retrospectively from 233 patients, treated with pentostatin ( n = 188) or cladribine ( n = 45), to investigate the current long-term outlook. Median follow-up was 16 years. There were no significant differences in outcome between the two agents. Overall, the complete response (CR) rate was 80% and median relapse-free survival was 16 years. After relapse ( n = 79) or non-response ( n = 5), 26 patients received pentostatin and 58 cladribine; 69% achieved CR and median relapse-free survival was 11 years. After third-line therapy ( n = 23), 50% achieved CR and median relapse-free survival was 6·5 years. However, CRs were equally durable, whether after first, second or third-line therapy. Complete responders and those with both haemoglobin >100 g/l and platelet count >100 × 109/l before treatment had the longest relapse-free survival ( P < 0·0001). Patients still in CR at 5 years had only a 25% risk of relapse by 15 years. Outcomes for patients with recurrent disease improved with the monoclonal antibody rituximab, combined with either purine analogue. Overall only eight patients died of HCL-related causes. Patients achieving a CR can expect a normal lifespan. |
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Keywords: | hairy cell leukaemia pentostatin cladribine rituximab survival |
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