Polycythemia Vera (PV) - Diagnosis and Management Summary

Diagnostic Criteria:

 • Elevated hemoglobin and/or hematocrit AND

 • Hypercellular bone marrow with trilineage growth AND

 • JAK2 mutation OR Abnormally low serum erythropoietin

1/2 of patients are discovered incidentally via CBC checked for an unrelated indication.  

When present, symptoms, signs, and complications can include:

 • "Vasomotor" symptoms (same as ET), Thrombosis, Hemorrhage, Pruritis, particularly following a warm bath or shower ("aquagenic pruritis"), Hypertension, Splenomegaly

Treatment:

 • As with ET, currently available treatments for PV are not curative, and do not prevent transformation into AML or MDS.

 • Treatment is for the reduction of symptoms and prevention of thrombosis.

 • Treatment algorithms are complicated and evolving, but very general options include:

    - Phlebotomy to target a hemoglobin concentration in the normal range

    - Phlebotomy + aspirin

    - Phlebotomy + hydroxyurea + aspirin

 • Aspirin should be avoided in patients with evidence of acquired von Willebrand disease



- Dr. Eric Strong @DrEricStrong - Strong Medicine https://www.youtube.com/c/EricsMedicalLectures/



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