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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]
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.
High platelet counts can occur in patients with polycythemia vera (high red blood cell counts), and is an additional risk factor for complications. [citation needed] A very small number of people report symptoms of erythromelalgia, a burning sensation and redness of the extremities that resolves with cooling, or aspirin or both. [3]
Hyperviscosity syndrome is a group of symptoms triggered by an increase in the viscosity of the blood. Symptoms of high blood viscosity include spontaneous bleeding from mucous membranes , visual disturbances due to retinopathy , and neurologic symptoms ranging from headache and vertigo to seizures and coma .
Hematological values such as hemoglobin, hematocrit, and red blood cell counts are elevated in patients with myomatous erythrocytosis syndrome. [3]The diagnostic criteria for myomatous erythrocytosis syndrome includes polycythemia, uterine myoma, and return of hemoglobin levels to normal following fibroid excision surgery.
For anticoagulant treatment, it is not clear if unfractionated and/or low molecular weight heparin treatment is effective at decreasing mortality and serious adverse events in this population. [19] There is also insufficient evidence to understand the risk of adverse effects associated with these treatment approaches in term or preterm infants.
Intrinsically related symptoms such as fever, malaise, pruritus (itching) due to the release of histamine, [5] fatigue, and right upper quadrant pain may be present in the afflicted patient. With some conditions, such as polycythemia vera, erythromelalgia, or burning of the palms and soles, coupled with thrombocytosis is common.
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]