title: Long-term follow-up and second malignancies in 487 patients with hairy cell leukaemia.
abstract: A large, multicentre, retrospective survey of patients with hairy cell leukaemia (HCL) was conducted in France to determine the frequency of second malignancies and to analyse the long-term effects of the established purine nucleoside analogues (PNAs), cladribine and pentostatin. The survey retrospectively reviewed the medical history of patients and their immediate family, clinical and biological presentation at the time of HCL diagnosis, treatment choice, response to treatment, time to relapse and cause of death. Data were collected for 487 patients with HCL. Of the patients included in the survey, 18% (88/487) had a familial history of cancers, 8% (41/487) presented with malignancies before HCL diagnosis and 10% (48/487) developed second malignancies after HCL was diagnosed. An excess incidence of second malignancies was observed, with a standardized incidence ratio (SIR) of 1·86 (95% confidence interval (CI): 1·34-2·51), with no significant difference between PNAs. For second haematological malignancies alone, the SIR was markedly increased at 5·32 (95% CI: 2·90-8·92). This study highlights the high frequency of cancers in HCL patients and their family members. The frequency of second malignancies is notably increased, particularly for haematological malignancies. The respective role of pentostatin and cladribine in the development of second malignancies is debatable.
authors.affiliation: Caen University Hospital, Laboratory of Haematology, Caen, France; University of Caen, Medical School, EA4652, Caen, France; Caen University Hospital, Regional Registry of Malignant Haemopathies of Lower-Normandy, Caen, France.
authors.forename: Edouard
authors.initials: E
authors.lastname: Cornet
authors.forename: Cécile
authors.initials: C
authors.lastname: Tomowiak
authors.forename: Aline
authors.initials: A
authors.lastname: Tanguy-Schmidt
authors.forename: Stéphane
authors.initials: S
authors.lastname: Lepretre
authors.forename: Jehan
authors.initials: J
authors.lastname: Dupuis
authors.forename: Pierre
authors.initials: P
authors.lastname: Feugier
authors.forename: Alain
authors.initials: A
authors.lastname: Devidas
authors.forename: Clara
authors.initials: C
authors.lastname: Mariette
authors.forename: Véronique
authors.initials: V
authors.lastname: Leblond
authors.forename: Catherine
authors.initials: C
authors.lastname: Thiéblemont
authors.forename: Patricia
authors.initials: P
authors.lastname: Validire-Charpy
authors.forename: Laurent
authors.initials: L
authors.lastname: Sutton
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Gyan
authors.forename: Jean-Claude
authors.initials: JC
authors.lastname: Eisenmann
authors.forename: Pascale
authors.initials: P
authors.lastname: Cony-Makhoul
authors.forename: Loïc
authors.initials: L
authors.lastname: Ysebaert
authors.forename: Xavier
authors.initials: X
authors.lastname: Troussard
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D057915:Drug Substitution; D005500:Follow-Up Studies; D005602:France / Q000453:epidemiology; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000453:epidemiology* / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D013156:Splenectomy; D016896:Treatment Outcome
keywords: hairy cell leukaemia; purine nucleoside analogues; second malignancies