TY - CHAP M1 - Book, Section TI - The Classic Myeloproliferative Neoplasms: Chronic Myelogenous Leukemia, Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis A1 - Tefferi, Ayalew A2 - Valle, David L. A2 - Antonarakis, Stylianos A2 - Ballabio, Andrea A2 - Beaudet, Arthur L. A2 - Mitchell, Grant A. PY - 2019 T2 - The Online Metabolic and Molecular Bases of Inherited Disease AB - The classic myeloproliferative neoplasms, including chronic myelogenous leukemia (CML), polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are a phenotypically diverse category of malignancies that are derived from stem cells in the myeloid lineage.The first genetic alteration recognized as a cause of myeloproliferative disease was the translocation that creates the BCR-ABL gene fusion. Recently, activating mutations in JAK2 and MPL have been found in the majority of BCR-ABL-negative myeloproliferative neoplasms. Recurrent mutations in these genes provide clinically useful diagnostic markers.Implicated by mutational evidence, the JAK-STAT pathway is a rational target for drug development. A number of anti-JAK2 drugs have undergone preclinical testing, and some of these have been introduced into clinical trials. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - ommbid.mhmedical.com/content.aspx?aid=1181418076 ER -