title: Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study.
abstract: Under the auspices of an International Working Group, seven centers submitted diagnostic and follow-up information on 1545 patients with World Health Organization-defined polycythemia vera (PV). At diagnosis, median age was 61 years (51% females); thrombocytosis and venous thrombosis were more frequent in women and arterial thrombosis and abnormal karyotype in men. Considering patients from the center with the most mature follow-up information (n=337 with 44% of patients followed to death), median survival (14.1 years) was significantly worse than that of the age- and sex-matched US population (P<0.001). In multivariable analysis, survival for the entire study cohort (n=1545) was adversely affected by older age, leukocytosis, venous thrombosis and abnormal karyotype; a prognostic model that included the first three parameters delineated risk groups with median survivals of 10.9-27.8 years (hazard ratio (HR), 10.7; 95% confidence interval (CI): 7.7-15.0). Pruritus was identified as a favorable risk factor for survival. Cumulative hazard of leukemic transformation, with death as a competing risk, was 2.3% at 10 years and 5.5% at 15 years; risk factors included older age, abnormal karyotype and leukocytes ≥15 × 10(9)/l. Leukemic transformation was associated with treatment exposure to pipobroman or P32/chlorambucil. We found no association between leukemic transformation and hydroxyurea or busulfan use.
authors.affiliation: Department of Medicine, Mayo Clinic, Rochester, MN, USA. tefferi.ayalew@mayo.edu
authors.forename: A
authors.initials: A
authors.lastname: Tefferi
authors.forename: E
authors.initials: E
authors.lastname: Rumi
authors.forename: G
authors.initials: G
authors.lastname: Finazzi
authors.forename: H
authors.initials: H
authors.lastname: Gisslinger
authors.forename: A M
authors.initials: AM
authors.lastname: Vannucchi
authors.forename: F
authors.initials: F
authors.lastname: Rodeghiero
authors.forename: M L
authors.initials: ML
authors.lastname: Randi
authors.forename: R
authors.initials: R
authors.lastname: Vaidya
authors.forename: M
authors.initials: M
authors.lastname: Cazzola
authors.forename: A
authors.initials: A
authors.lastname: Rambaldi
authors.forename: B
authors.initials: B
authors.lastname: Gisslinger
authors.forename: L
authors.initials: L
authors.lastname: Pieri
authors.forename: M
authors.initials: M
authors.lastname: Ruggeri
authors.forename: I
authors.initials: I
authors.lastname: Bertozzi
authors.forename: N H
authors.initials: NH
authors.lastname: Sulai
authors.forename: I
authors.initials: I
authors.lastname: Casetti
authors.forename: A
authors.initials: A
authors.lastname: Carobbio
authors.forename: G
authors.initials: G
authors.lastname: Jeryczynski
authors.forename: D R
authors.initials: DR
authors.lastname: Larson
authors.forename: L
authors.initials: L
authors.lastname: Müllauer
authors.forename: A
authors.initials: A
authors.lastname: Pardanani
authors.forename: J
authors.initials: J
authors.lastname: Thiele
authors.forename: F
authors.initials: F
authors.lastname: Passamonti
authors.forename: T
authors.initials: T
authors.lastname: Barbui
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002471:Cell Transformation, Neoplastic; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D015994:Incidence; D007938:Leukemia / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011087:Polycythemia Vera / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D011379:Prognosis; D012372:ROC Curve; D055815:Young Adult