Polycythemia Vera (PV) - Diagnosis and Management Summary
• 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
• 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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