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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

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
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
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 x 10(9)/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.
0007-1048
733-740
Else, Monica
3d354259-1969-4d47-9eea-5b95daaf2089
Dearden, Claire E.
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Matutes, Estella
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Garcia-Talavera, Juan
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Rohatiner, Ama Z.S.
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Johnson, Steve A.N.
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O'Connor, Nigel T.J.
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Haynes, Andy
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Osuji, Nnenna
b8eef79e-1890-4f8b-a4d7-2738d9db1afb
Forconi, Francesco
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Lauria, Francesco
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Catovsky, Daniel
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Else, Monica
3d354259-1969-4d47-9eea-5b95daaf2089
Dearden, Claire E.
7bf313f7-6e4e-4660-a1a1-65cfd21afb9f
Matutes, Estella
3da6561d-9c65-48b3-9273-db3c93f77a80
Garcia-Talavera, Juan
f9efbb00-e992-4e63-bdf8-38b6aa31b801
Rohatiner, Ama Z.S.
cbad88d3-2231-4d20-a236-9da6d127e204
Johnson, Steve A.N.
b1458465-a077-4995-9b42-4632d3373648
O'Connor, Nigel T.J.
06c82a89-79a9-40db-a83c-2a04bc7ac29b
Haynes, Andy
8f333c3a-296c-43a3-a0d7-46c36bebe0ee
Osuji, Nnenna
b8eef79e-1890-4f8b-a4d7-2738d9db1afb
Forconi, Francesco
ce9ed873-58cf-4876-bf3a-9ba1d163edc8
Lauria, Francesco
f01f163b-abcb-4aad-b952-f4356692f519
Catovsky, Daniel
0c2a5c78-d841-456e-88c7-581850d4e80a

Else, Monica, Dearden, Claire E., Matutes, Estella, Garcia-Talavera, Juan, Rohatiner, Ama Z.S., Johnson, Steve A.N., O'Connor, Nigel T.J., Haynes, Andy, Osuji, Nnenna, Forconi, Francesco, Lauria, Francesco and Catovsky, Daniel (2009) 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. British Journal of Haematology, 145 (6), 733-740. (doi:10.1111/j.1365-2141.2009.07668.x). (PMID:19344416)

Record type: Article

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 x 10(9)/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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e-pub ahead of print date: 29 March 2009
Published date: June 2009
Organisations: Cancer Sciences

Identifiers

Local EPrints ID: 358154
URI: http://eprints.soton.ac.uk/id/eprint/358154
ISSN: 0007-1048
PURE UUID: 8c5a356c-38ed-43ac-a883-125923340b41
ORCID for Francesco Forconi: ORCID iD orcid.org/0000-0002-2211-1831

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Date deposited: 08 Oct 2013 13:03
Last modified: 15 Mar 2024 03:41

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Contributors

Author: Monica Else
Author: Claire E. Dearden
Author: Estella Matutes
Author: Juan Garcia-Talavera
Author: Ama Z.S. Rohatiner
Author: Steve A.N. Johnson
Author: Nigel T.J. O'Connor
Author: Andy Haynes
Author: Nnenna Osuji
Author: Francesco Lauria
Author: Daniel Catovsky

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