Patterson-Fortin Jeffrey, Moliterno Alison R
Department of Medicine, The Johns Hopkins University School of Medicine, 600 North Wolfe Street, Nelson-Harvey 808, Baltimore, MD, 21287, USA.
Division of Hematology, Department of Medicine, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, Ross Building, #1025, Baltimore, MD, 21205, USA.
Curr Hematol Malig Rep. 2017 Oct;12(5):424-431. doi: 10.1007/s11899-017-0411-0.
The myeloproliferative neoplasms polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) display distinct clinical and pathologic features but are characterized by mutations in JAK2, MPL, and CALR leading to activation of the JAK-STAT pathway. This review addresses the pathogenesis and mechanisms of these mutant alleles and the unique interactions of both of age and gender.
骨髓增殖性肿瘤真性红细胞增多症(PV)、原发性血小板增多症(ET)和原发性骨髓纤维化(PMF)表现出不同的临床和病理特征,但均以JAK2、MPL和CALR突变导致JAK-STAT通路激活为特征。本综述探讨了这些突变等位基因的发病机制以及年龄和性别的独特相互作用。