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Prognosis and treatment of polycythemia vera

Ayalew Tefferi, MD
Section Editor
Stanley L Schrier, MD
Deputy Editor
Alan G Rosmarin, MD


Polycythemia vera (PV) is one of the myeloproliferative neoplasms, which are collectively characterized by clonal proliferation of myeloid cells with variable morphologic maturity and hematopoietic efficiency (table 1) [1]. PV is distinguished clinically from the other myeloproliferative neoplasms by the presence of an elevated red blood cell mass (RCM, also called erythrocytosis) (table 2). (See "Overview of the myeloproliferative neoplasms".)

This topic will review the prognosis and treatment of polycythemia vera [2-4]. Approaches to the patient with suspected polycythemia vera or polycythemia in general are discussed separately. (See "Clinical manifestations and diagnosis of polycythemia vera" and "Diagnostic approach to the patient with polycythemia".)


The median survival of untreated symptomatic patients with PV was initially estimated at 6 to 18 months from the time of diagnosis [5], whereas current survival of treated patients is 13 years or more [6]. Even with treatment, overall mortality is greater than that of an age- and sex-matched normal population [6-9]. In a large multinational prospective study of 1638 patients, the overall mortality rate was 3.7 deaths per 100 persons/year [10]. Cardiovascular mortality, solid tumors, and hematologic transformation accounted for 45, 20, and 13 percent of the deaths, respectively.

Other prospective data were obtained from the Polycythemia Vera Study Group (PVSG) major protocol of over 400 patients, all of whom were treated [11]. The median survival varied from 9.1 to 12.6 years with different therapies. The most common causes of death were thrombosis (29 percent), hematologic malignancies (23 percent), nonhematologic malignancies (16 percent), hemorrhage (7 percent), and myelofibrosis/myeloid metaplasia (3 percent).

A long-term study using registry data from two well-defined cohorts evaluated prognostic risk factors for survival and leukemia in 327 PV patients. At a median follow-up of 11 years, the following observations were made [12]:


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