Ronald Hoffman, MD, Director of the Myeloproliferative Diseases Program at The Icahn School of Medicine at Mount Sinai, describes the typical treatment options for polycythemia vera.
Polycythemia vera, as outlined by the NIH, is a rare blood disorder characterized by increased concentration of blood cells. Most cases of polycythemia vera that are acquired afflict older persons, but it is associated with mutations in various genes, including the JAK2 gene.
As Dr. Hoffman explains, the treatment for low-risk polycythemia vera patients is intermittent phlebotomy therapy and low dose aspirin therapy. The typical treatment for high-risk patients is a variety of agents, such as hydroxyurea or pegylated interferon in addition to supplemental phlebotomy therapy. For high-risk older patients who cannot tolerate phlebotomy, intermittent therapy with alkylating agents such as busulfan or melphalan. According to Dr. Hoffman, the only time a physician should be concerned about an elevation of platelet count in polycythemia vera patients is if said patient develops acquired von Willebrand syndrome, which is associated with severe bleeding episodes.
For more information about polycythemia vera and other rare hematologic disorders, visit https://checkrare.com/diseases/hematologic-disorders/