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The most common therapies for secondary polycythemia are phlebotomies [9] and, for paraganglioma and/or somatostatinoma in this cohort of patients, surgery accompanied by antihypertensive medication. [1] HIF-2α inhibitor belzutifan led to substantial improvement of symptoms in a patient with Pacak–Zhuang syndrome. [10]
Treatment can include phlebotomy, aspirin, and myelosuppressive or cytoreductive medications based on risk stratification. [7] For secondary polycythemia, management involves addressing the underlying etiology of increased erythropoeitin production, such as smoking cessation, CPAP for sleep apnea, or removing any EPO-producing tumours. [6]
It is indicated for the treatment of intermediate or high-risk myelofibrosis, including primary myelofibrosis or secondary myelofibrosis [post-polycythemia vera and post-essential thrombocythemia], in adults with anemia. [31] [32]
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.
polycythemia ruba vera is secondary polycythemia and has an underlying cause such as sleep apnea copd or anything that causes a rise in epo and is treatable by treating the underlying cause. polycythemia vera known as primary polycythemia has no secondary causes that can be found your oxygen levels are normal and you epo is normal or low.
289.6 Familial polycythemia; 289.7 Methemoglobinemia; 289.8 Other specified diseases of blood and blood-forming organs 289.81 Primary hypercoagulable state; 289.82 Secondary hypercoagulable state; 289.83 Myelofibrosis; 289.84 Heparin-induced thrombocytopenia; 289.89 Other specified diseases of blood and blood-forming organs
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B3. No evidence of polycythemia vera. hematocrit < midpoint of normal range or normal red cell mass in presence of normal iron stores; B4. No evidence of chronic myeloid leukemia. But the Philadelphia chromosome may be present in up to 10% of cases.
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