title: Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study.
abstract: Progression to acute myeloid leukemia/myelodysplastic syndromes (AML/MDS) is a possible evolution of polycythemia vera (PV), but whether some patients are at increased natural risk for this complication and how much the contribution of pharmacologic cytoreduction can affect the natural course of the disease remain uncertain. The European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) prospective project included 1638 patients with PV. AML/MDS was diagnosed in 22 patients after a median of 2.5 years from recruitment in the study and a median of 8.4 years from the diagnosis of PV. Variables associated with progression to AML/MDS were assessed using different models of multivariate analysis. Older age was confirmed as the main independent risk factor (hazard ratio [HR], 4.30; 95% confidence interval [95% CI], 1.16-15.94; P = .0294), whereas overall disease duration failed to reach statistical significance (more than 10 years: HR, 1.91; 95% CI, 0.64-5.69; P = .2466). Exposure to P32, busulphan, and pipobroman (HR, 5.46; 95% CI, 1.84-16.25; P = .0023), but not to hydroxyurea (HU) alone (HR, 0.86; 95% CI, 0.26-2.88; P = .8021), had an independent role in producing an excess risk for progression to AML/MDS compared with treatment with phlebotomy or interferon.
authors.affiliation: Consorzio Mario Negri Sud, Santa Maria Imbaro, Italy.
authors.forename: Guido
authors.initials: G
authors.lastname: Finazzi
authors.forename: Vanesa
authors.initials: V
authors.lastname: Caruso
authors.forename: Roberto
authors.initials: R
authors.lastname: Marchioli
authors.forename: Giovanni
authors.initials: G
authors.lastname: Capnist
authors.forename: Teodoro
authors.initials: T
authors.lastname: Chisesi
authors.forename: Carlo
authors.initials: C
authors.lastname: Finelli
authors.forename: Luigi
authors.initials: L
authors.lastname: Gugliotta
authors.forename: Raffaele
authors.initials: R
authors.lastname: Landolfi
authors.forename: Jack
authors.initials: J
authors.lastname: Kutti
authors.forename: Heinz
authors.initials: H
authors.lastname: Gisslinger
authors.forename: Raphael
authors.initials: R
authors.lastname: Marilus
authors.forename: Carlo
authors.initials: C
authors.lastname: Patrono
authors.forename: Enrico Maria
authors.initials: EM
authors.lastname: Pogliani
authors.forename: Maria Luigia
authors.initials: ML
authors.lastname: Randi
authors.forename: Ana
authors.initials: A
authors.lastname: Villegas
authors.forename: Gianni
authors.initials: G
authors.lastname: Tognoni
authors.forename: Tiziano
authors.initials: T
authors.lastname: Barbui
mesh_terms: D000208:Acute Disease; D000328:Adult; D000368:Aged; D001241:Aspirin / Q000627:therapeutic use; D016208:Databases, Factual; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007951:Leukemia, Myeloid / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011087:Polycythemia Vera / Q000188:drug therapy / Q000453:epidemiology*; D011446:Prospective Studies; D012307:Risk Factors
chemical_list: D010975:Platelet Aggregation Inhibitors; D001241:Aspirin