Polycythemia vera

Abstract

The diagnostic approach to a patient with polycythemia has been greatly simplified by the introduction of new genetic testing in addition to traditional tests, such as measurement of red cell mass and serum erythropoietin (Epo) level. Clonal erythrocytosis, which is the diagnostic feature of polycythemia vera (PV), is almost always associated with a JAK2 mutation (JAK2V617F or exon 12). Therefore, in a patient with acquired erythrocytosis, it is reasonable to begin the diagnostic work-up with JAK2 mutation analysis to distinguish PV from secondary erythrocytosis. The clinical course of PV is marked by a high incidence of thrombotic complications that represent the main cause of morbidity and mortality in these patients. Blood hyperviscosity as well as platelet and leukocyte quantitative, and qualitative abnormalities play a major role in the pathogenesis of thrombophilia. Prevention of vascular events and minimizing the risk of disease transition into acute leukaemia are the main targets of the whole PV treatment strategy. This can rely on the use of low-dose aspirin in most patients, while the choice of the optimal cytoreductive strategy is based on the individual vascular risk. Phlebotomy is still the preferred treatment in subjects at low risk, while hydroxyurea or pipobroman is usually administered to most elderly subjects or subjects with a previous vascular history. The use of pegylated interferon, imatinib, and JAK2 inhibitors is currently being evaluated.

Treatment outcome in a cohort of young patients with polycythemia vera

“…However, the use of myelosuppressive agents (in particular alkylating agents) to treat high-risk patients should be carefully balanced against the possible increased risk of cancer evolution, and, whenever necessary, hydroxyurea should be preferred [11,12]. Moreover, in young, asymptomatic patients with PV, as in patients with a diagnosis of Essential Thrombocythemia [13,14], a high platelet count can be left untreated, considering carefully the use of cytoreduction.…”

Nursing Care in Polycythemia Vera: A Review Article

“…The "Autar DVT Risk Diagnosis Scale", the use of which is suitable for polycythemia patients, can be used in order to determine the risk of DVT [17]. The risk of bleeding due to the use of high doses of aspirin and/or acquired von Willebrand Disease, increases in PV [6,7,18]. In order for bleeding to be able to be detected early and the necessary precautions to be taken in a timely manner in clinics, the risk of bleeding and bleeding should be evaluated based on the NCI-CTCAE version 4.03 classification criteria [19].…”

Subfoveal Choroidal Thickness Is Associated with Blood Hematocrit Level

“…Polycythemia vera (PV) is a rare chronic myeloproliferative disorder characterized by abnormally increased production of blood cells, particularly red blood cells in the bone marrow [1]. The annual incidence rate of PV is estimated to be approximately 2 cases per 100,000 persons in Europe and the United States [1].…”

“…The annual incidence rate of PV is estimated to be approximately 2 cases per 100,000 persons in Europe and the United States [1]. The risk of thrombosis in PV exceeds 20%, and a significant number of patients have vasomotor disturbances such as headaches [2].…”