Acute leukemia in polycythemia vera: an analysis of 1638 patients enrolled in a prospective observational study

• Published in: Blood 2005-04, Vol.105 (7), p.2664-2670
• Main Authors: Finazzi, Guido, Caruso, Vanesa, Marchioli, Roberto, Capnist, Giovanni, Chisesi, Teodoro, Finelli, Carlo, Gugliotta, Luigi, Landolfi, Raffaele, Kutti, Jack, Gisslinger, Heinz, Marilus, Raphael, Patrono, Carlo, Pogliani, Enrico Maria, Randi, Maria Luigia, Villegas, Ana, Tognoni, Gianni, Barbui, Tiziano
• Format: Article
• Language: English
• Subjects: Acute Disease; Adult; Aged; Aspirin - therapeutic use; Biological and medical sciences; Databases, Factual; Disease Progression; Disease-Free Survival; Female; Hematologic and hematopoietic diseases; Humans; Leukemia, Myeloid - epidemiology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Male; Medical sciences; Middle Aged; Multivariate Analysis; Platelet Aggregation Inhibitors - therapeutic use; Polycythemia Vera - drug therapy; Polycythemia Vera - epidemiology; Prospective Studies; Risk Factors
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2004-09-3426

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.