title: A multicentric study of 41 cases of B-prolymphocytic leukemia: two evolutive forms.
abstract: B-prolymphocytic leukemia (B-PLL) is an infrequent disease with a poor prognosis. We present the clinical and biological features of 41 patients. Median age was 67 years [42-89] and male-female sex ratio was 2.4. The immunophenotyping revealed B-cell phenotype, with a high level expression of surface IgM and/or IgD in all cases, FMC7+ in 76 % of cases and CD5+ in 67%. Marked spontaneous in-vitro apoptosis was observed in most cases tested (n = 12). The median overall survival time was 5 years and the event-free survival time was 37 months. As detected by univariate and multivariate analysis, the only variables associated with a poor prognosis were advanced age and anemia. No significant difference was observed between de novo PLL (n = 27) and prolymphocytoid transformation of chronic lymphocytic leukemia (n = 14). Two groups of patients were individualized according to their clinical course: patients who died within one year of diagnosis (n = 14) and patients who had a prolonged survival (n = 23) without any treatment in some cases. The comparison between the 2 groups showed that they differed in age (p = 0.01) and anemia (p = 0.02). We also observed that the patients with p53 mutations had a worse clinical outcome. Taken together these data confirm that B-PLL should be regarded as a distinct form of chronic lymphoproliferative disorder and suggest the existence of two patterns of clinical evolution.
authors.affiliation: Service d'Hématologie Biologique, C.H.U. Pitié-Salpêtrière Hospital, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Hercher
authors.forename: M
authors.initials: M
authors.lastname: Robain
authors.forename: F
authors.initials: F
authors.lastname: Davi
authors.forename: R
authors.initials: R
authors.lastname: Garand
authors.forename: G
authors.initials: G
authors.lastname: Flandrin
authors.forename: F
authors.initials: F
authors.lastname: Valensi
authors.forename: H
authors.initials: H
authors.lastname: Vandeputte
authors.forename: A
authors.initials: A
authors.lastname: Albert
authors.forename: M
authors.initials: M
authors.lastname: Maynadie
authors.forename: X
authors.initials: X
authors.lastname: Troussard
authors.forename: G H
authors.initials: GH
authors.lastname: Simon
authors.forename: J
authors.initials: J
authors.lastname: Lespinasse
authors.forename: G
authors.initials: G
authors.lastname: Portefaix
authors.forename: H
authors.initials: H
authors.lastname: Merle-Beral
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000740:Anemia / Q000209:etiology; D017209:Apoptosis; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000145:classification* / Q000175:diagnosis / Q000473:pathology; D015463:Leukemia, Prolymphocytic / Q000145:classification* / Q000175:diagnosis / Q000473:pathology; D017254:Leukemic Infiltration; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis