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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.
[15] [16] [17] Patients are usually designated as having a low or high risk of bleeding or developing blood clots based on their age, medical history, blood counts and their lifestyles. Low risk individuals are usually treated with aspirin , whereas those at high risk are treated with hydroxycarbamide , interferon-α or anagrelide ).
Conditioning treatment before haematopoietic stem cell transplantation (high dose, IV), chronic myeloid leukaemia, myelofibrosis, polycythaemia vera and essential thrombocytosis Myelosuppression, seizures (high dose), tachycardia (high dose), hepatic sinusoidal obstruction syndrome (high dose), Addison-like syndrome (rare), pulmonary fibrosis ...
Polycythemia vera (PCV) (a.k.a. polycythemia rubra vera (PRV)) occurs when excess red blood cells are produced as a result of an abnormality of the bone marrow. [3] Often, excess white blood cells and platelets are also produced. A hallmark of polycythemia vera is an elevated hematocrit, with Hct > 55% seen in 83% of cases. [19]
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]
Polycythemia vera (PV) is associated most often with the JAK2 V617F mutation greater than 95% of cases, whereas the remainder has a JAK2 exon 12 mutations. High hemoglobin or hematocrit counts are required, as is a bone marrow examination showing "prominent erythroid, granulocytic and megakaryocytic proliferation with pleomorphic, mature ...
[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]
However, splenectomy in the presence of massive splenomegaly is a high-risk procedure, with a mortality risk as high as 3% in some studies. [23] In November 2011, the US Food and Drug Administration (FDA) approved ruxolitinib (Jakafi) as a treatment for intermediate or high-risk myelofibrosis. [24] [25] Ruxolitinib serves as an inhibitor of JAK ...
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