Barbui and Finazzi used a flow diagram to demonstrate the choice of therapeutic interventions for polycythemia rubra vera based on risk factors in the specific patient. This was published in 1997 and the selection of optimum therapy will be affected by subsequent introduction of new regimens.


All patients should be phlebotomized to keep the hematocrit <= 45 percent.


All patients should be considered for aspirin prophylaxis (or other antiplatelet agent if aspirin cannot be tolerated).

• A patient with a history of thrombotic episode is at high risk for further thrombotic events and so needs to be more aggressively treated.

• A person > 70 years may benefit from low dose aspirin therapy for prophylaxis.


Further therapy depends on:

(1) age

(2) evidence of high risk disease (presentation with thrombosis OR high rate of phlebotomy OR platelet count > 400,000 per µL)

(3) concern about secondary leukemias and cancer related to use of phosphorus-32 and agents like chlorambucil.


If the patient is > 70, then the patient may receive myelosuppressive therapy (with phosphorus-32 or busulfan).


If the patient has high risk disease and from 40-70 years of age, then the patient may receive myelosuppression (with hydroxyurea).


If the patient has high risk disease and < 40 years of age, then consider therapy with interferon alpha or anagrelide (an agent that reduces platelet production and aggregation).


If the patient is <= 70 and not in high risk group, then the choice may be to not give myelosuppression initially, but to reconsider if complications occur.


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