Originally published as JCO Early Release 10.1200/JCO.2005.07.062 on February 14 2005

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Vascular and Neoplastic Risk in a Large Cohort of Patients With Polycythemia Vera

From the Consorzio Mario Negri Sud, Santa Maria Imbaro; the Catholic University School of Medicine, and the University of Rome "La Sapienza," Rome; the Ospedali Riuniti, Bergamo, Italy; the Sahlgrenska Hospital, Göteborg, Sweden; the Department of Hematology and Blood Coagulation, University of Vienna, Austria; the Tel-Aviv Souraski Medical Center, Tel-Aviv, Israel; and the Hospital Universitario S Carlos, Madrid, Spain

Address reprint requests to R. Marchioli, European Collaboration on Low-Dose Aspirin in Polycythemia Vera Coordinating Centre, Consorzio Mario Negri Sud, Via Nazionale, 66030 Santa Maria Imbaro, Italy; e-mail: marchioli{at}negrisud.it

PURPOSE: The clinical course of polycythemia vera is often complicated by thrombosis as well as by the possible transition to myeloid metaplasia with myelofibrosis or acute myeloid leukemia. The aim of this study was to assess the rate of these complications in subjects receiving currently recommended treatments.

PATIENTS AND METHODS: Overall, 1,638 patients from 12 countries were enrolled onto a large, prospective multicenter project aimed at describing the clinical history of polycythemia vera for the following outcomes: survival, the cumulative rate of cardiovascular death and thrombosis, the cumulative rate of leukemia, myelodysplasia, and myelofibrosis. The mean duration of the disease at entry and the duration of the follow-up were 4.9 and 2.7 years, respectively.

RESULTS: The overall mortality rate of 3.7 deaths per 100 persons per year resulted from a moderate risk of cardiovascular death and a high risk of death from noncardiovascular causes (mainly hematologic transformations). Age older than 65 years and a positive history of thrombosis were the most important predictors of cardiovascular events. Antiplatelet therapy, but not cytoreductive treatment, was significantly associated with a lower risk of cardiovascular events. We found a consistent association between age and risk of leukemia, and between duration of the disease with risk of myelofibrosis.

CONCLUSION: The European Collaboration on Low-Dose Aspirin in Polycythemia Vera study documents that large international collaborative studies are feasible in this field, in which few epidemiologic data are available. The persistently high mortality rate from hematologic malignancies characterizes the unmet therapeutic need of polycythemic patients and suggests a priority for future studies in this disease.

Supported by the European Union BIOMED 2 Program (contract No. ERBBMH4CT961433).

Presented at the 45th Annual Meeting of the American Society of Hematology, San Diego, CA, December 6-9, 2003.

The European Collaboration on Low-Dose Aspirin in Polycythemia Vera Investigators are listed in the Appendix.

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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