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In oncology, polycythemia vera (PV) is an uncommon myeloproliferative neoplasm in which the bone marrow makes too many red blood cells. [1] The majority of cases [ 2 ] are caused by mutations in the JAK2 gene, most commonly resulting in a single amino acid change in its protein product from valine to phenylalanine at position 617.
[3] [6] Ropeginterferon alfa-2b is the first medication approved by the US Food and Drug Administration (FDA) to treat polycythemia vera that people can take regardless of their treatment history, and the first interferon therapy specifically approved for polycythemia vera. [3] The FDA considers it to be a first-in-class medication. [7]
Treatment for polycythemia varies, and typically involves treating its underlying cause. [6] Treatment of primary polycythemia (see polycythemia vera) could involve phlebotomy, antiplatelet therapy to reduce risk of blood clots, and additional cytoreductive therapy to reduce the number of red blood cells produced in the bone marrow. [7]
A therapeutic phlebotomy may be carried out in the treatment of some blood disorders (example: Hemochromatosis, polycythemia vera, porphyria cutanea tarda), and chronic hives (in research). [ 8 ] [ 9 ]
Therapeutic phlebotomy refers to the drawing of a unit of blood in specific cases like hemochromatosis, polycythemia vera, porphyria cutanea tarda, etc., to reduce the number of red blood cells. [ 6 ] [ 7 ] The traditional medical practice of bloodletting is today considered to be a pseudoscience .
A third Imago-sponsored global clinical trial for the treatment of essential thrombocythemia was begun in 2020. [10] The results of this global study have also been presented at the American Society of Hematology and the European Hematology Association. A fourth trial begun in 2023 for the treatment of polycythemia vera is ongoing. [11]
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Hydroxycarbamide, also known as hydroxyurea, is an antimetabolite medication used in sickle-cell disease, essential thrombocythemia, chronic myelogenous leukemia, polycythemia vera, and cervical cancer. [4] [5] In sickle-cell disease it increases fetal hemoglobin and decreases the number of attacks. [4] It is taken by mouth. [4]