Optimal management includes the following.
1. Venesection, with isovolemic replacement using crystalloids or colloids to reduce the hematocrit to less than 0.50, and preferably to about 0.45, is used to correct the blood hyperviscosity associated with the erythrocytosis of untreated polycythemia vera.
2. At the same time as the venesection is started, hydroxyurea is given in a dose ranging from 500 mg twice daily to 500 mg five times daily to control hemopoiesis over the first 10 to 14 days of treatment.
3. If more rapid effects are needed to reduce the thrombocythemia when the patient has presented with organ ischemia associated with platelet counts in excess of 750 x i09/l, cytarabine 0.5 g can be given intravenously on one or two occasions for acute curtailment of platelet production during the following 2 to 7 days. Reduction of circulating platelet counts by platelet pheresis is ineffective and is not worthwhile.
4. Platelet function can also be reduced using aspirin in a dose of 150 mg on alternate days.
5. Once the diagnosis is confirmed as primary polycythemia in an adult over 60 years of age, radio-phosphorus (32P) 185 mBq (6 mCi) is given intravenously for longer-term control, which lasts for months to 2 years in most patients. Radio-phosphorus is the definitive treatment for such patients. Most patients presenting with polycythemia rubra vera or thrombocythemia may have a normal lifespan because of the success of this treatment in maintaining remission for many years.
6. In younger patients, control of the myeloproliferation is possible using hydroxyurea in a maintenance dose of 1 to 2 g daily, with out-patient clinic review every few weeks or months depending on the individual patient's response. The long-term risks of this treatment seem to be less than those associated with alkylating agents (e.g. bulsulphan), but the latter may be useful in an initial dose of 2 to 6 mg daily if hydroxyurea is not well tolerated.
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