TY - CHAP M1 - Book, Section TI - Polycythemia Vera A1 - Lichtman, Marshall A. A1 - Kaushansky, Kenneth A1 - Prchal, Josef T. A1 - Levi, Marcel M. A1 - Burns, Linda J. A1 - Linch, David C. PY - 2022 T2 - Williams Manual of Hematology, 10e AB - Polycythemia vera (PV) is a clonal disorder arising from somatic mutations of a hematopoietic stem, or closely related multipotential cell, in which blood cell production, notably in the erythroid lineage, but also in the granulocytic, monocytic, and platelet lineages, is increased and is independent of cytokine regulation. This results in exaggerated proliferation and accumulation of erythrocytic, and often granulocytic and megakaryocytic, precursors in the marrow and their mature cells in the blood. PV is one of the myeloproliferative neoplasms (MPNs), along with essential thrombocythemia (ET), primary myelofibrosis (MF), and chronic myelogenous leukemia (CML).Three MPNs (PV, ET [Chap. 43], and MF [Chap. 48]) may share a common molecular abnormality/marker, the JAK2 kinase V617F mutation. In contrast, CML is caused by a different molecular alteration, the BCR/ABL oncogene, due to a reciprocal translocation between chromosome 9 and chromosome 22, t(9;22)(q34;q11), and the production of the BCR-ABL protein product (Chap. 47). SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/18 UR - hemonc.mhmedical.com/content.aspx?aid=1189334421 ER -