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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.
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]
As reported by Dispenzieri et al. [4] Mayo Clinic treatment regimens are tailored to treat the clinical manifestations and prognosis for the rate of progression of the POEMS syndrome in each patient. In rare cases, patients may have minimal or no symptoms at presentation or after successful treatment of their disorder.
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.
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]
In hematology, plasma cell dyscrasias (also termed plasma cell disorders and plasma cell proliferative diseases) are a spectrum of progressively more severe monoclonal gammopathies in which a clone or multiple clones of pre-malignant or malignant plasma cells (sometimes in association with lymphoplasmacytoid cells or B lymphocytes) over-produce and secrete into the blood stream a myeloma ...
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.
Twin anemia-polycythemia sequence (TAPS) is a chronic type of unbalanced fetal transfusion in monochorionic twins that results in polycythemia in the TAPS recipient and anemia in the TAPS donor due to tiny placental anastomoses. [1] Post-laser TAPS and spontaneous TAPS are the two forms of TAPS.
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