Epidemiology

The reported incidence of PV varies from as low as 0.2 cases per million in Japan4 to around 20 cases per million in Minnesota,5 with most reports suggesting an incidence of between 5 and 10 cases per million population per year.6-8 Because the disease is chronic, its prevalence is considerably higher. The wide variation in disease incidence may be explained by a differing predisposition toward PV among certain racial and ethnic backgrounds, with Ashkenazi Jews residing in the United States and Israel having a fourfold increase in incidence by comparison with the remainder of the population7-9 and African Americans affected less frequently than the remainder of the population of the United States.10,11 An increased incidence of PV has been reported following exposure to ionizing radiation, with survivors of the atomic bomb detonation in Hiroshima having a higher incidence compared with the rest of the Japanese popu-lation,12 and U.S. military personnel involved with the detonation of an atomic device having a 20-fold incidence compared with the general population.13 The sex incidence is roughly equivalent with slight male predominance. PV occurs mainly in late middle age and the elderly. In a large retrospective study from the Gruppo Italiano Studio Policitemia14 involving more than 1000 patients the median age at diagnosis was 60 years. Younger patients are seen, however, with 7 percent presenting before age 40 in this study.

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