Natural history and treatment

• Not clear. Retrospective analysis appears to suggest an 4 incidence of vaso-occlusive episodes that may relate to associated risk factors in the lifestyle of the patients under observation (rather than the 4 PCV).

• Low dose aspirin (75mg/d) advisable for patients with overt thrombotic risks and no GI contraindication.

• No role for myelosuppressive therapy.

• Many patients improve with the simple measures specified. In ~33% the Hct returns to the normal range. In a further 33% the Hct oscillates between minimal elevation and the normal range. In the remaining 33% the Hct remains elevated and these patients should receive long term follow-up. In a minority absolute erythrocytosis may develop.

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