title: Hairy cell leukemia: evaluation of the long-term outcome in 121 patients.
abstract: BACKGROUND
  Historically, the first treatment choices for hairy cell leukemia (HCL) were splenectomy and alpha-interferon. Recently, purine analogues (pentostatin and cladribine) changed radically the treatment modality, inducing complete and durable responses in the majority of patients.
  
  
  METHODS
  The authors analyzed the outcome of different lines of therapy in 121 HCL patients followed in their institute from 1986 to 2008, with a median follow-up of 105 months. Patients were divided into subgroups according to the number of treatments; Group A included 121 patients who underwent a front-line therapy, Group B patients (n =53) were treated with 2 lines, Group C patients (n = 34) with 3 lines, Group D patients (n = 17) with 4 lines, and Group E patients (n = 8) with 5 lines.
  
  
  RESULTS
  In Group A, 92 (77%) patients obtained a complete response (CR), 23 (18%) a partial response, and the remaining 6 (5%) a minor or no response; median duration of response was 2.7 years. In Group B, 53 relapsed patients achieved a second CR rate of 73.5%; median duration of response was 2.5 years. Group C contained 34 patients in a second relapse, with a CR rate after the third line of treatment of 70.5% (median duration of response, 2.2 years). In Group D, 11 (64.7%) patients obtained a CR (median duration of response, 1.6 years), and in Group E 4 (50%) of 8 patients achieved a CR (median duration of response, 1.3 years).
  
  
  CONCLUSIONS
  This study confirms the high risk (>40% of all patients) of retreatment of HCL patients and the need to maximize primary response.
authors.affiliation: Institute of Hematology and Medical Oncology "L. e A. Seragnoli," University of Bologna, Bologna, Italy. pierluigi.zinzani@unibo.it
authors.forename: Pier Luigi
authors.initials: PL
authors.lastname: Zinzani
authors.forename: Cinzia
authors.initials: C
authors.lastname: Pellegrini
authors.forename: Vittorio
authors.initials: V
authors.lastname: Stefoni
authors.forename: Enrico
authors.initials: E
authors.lastname: Derenzini
authors.forename: Letizia
authors.initials: L
authors.lastname: Gandolfi
authors.forename: Alessandro
authors.initials: A
authors.lastname: Broccoli
authors.forename: Lisa
authors.initials: L
authors.lastname: Argnani
authors.forename: Federica
authors.initials: F
authors.lastname: Quirini
authors.forename: Stefano
authors.initials: S
authors.lastname: Pileri
authors.forename: Michele
authors.initials: M
authors.lastname: Baccarani
mesh_terms: D015762:2-Chloroadenosine / Q000031:analogs & derivatives* / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use*; D003839:Deoxyadenosines / Q000627:therapeutic use*; D018572:Disease-Free Survival; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007943:Leukemia, Hairy Cell / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D019233:Retreatment; D013156:Splenectomy; D016896:Treatment Outcome
chemical_list: D000964:Antimetabolites, Antineoplastic; D003839:Deoxyadenosines; D015762:2-Chloroadenosine; C055994:2-chloro-3'-deoxyadenosine