Hematology Division, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD; and.
Division of Hematology and Medical Oncology and.
Blood. 2021 Mar 4;137(9):1145-1153. doi: 10.1182/blood.2020008043.
Philadelphia chromosome-negative myeloproliferative neoplasms (MPNs), polycythemia vera, essential thrombocythemia, and primary myelofibrosis, are hematopoietic stem cell disorders that are defined by activating mutations in signal transduction pathways and are characterized clinically by the overproduction of platelets, red blood cells, and neutrophils, significant burden of disease-specific symptoms, and high rates of vascular events. The focus of this review is to critically reevaluate the clinical burden of thrombosis in MPNs, to review the clinical associations among clonal hematopoiesis, JAK2V617F burden, inflammation, and thrombosis, and to provide insights into novel primary and secondary thrombosis-prevention strategies.
费城染色体阴性骨髓增殖性肿瘤（MPN），包括真性红细胞增多症、原发性血小板增多症和原发性骨髓纤维化，是由信号转导通路的激活突变定义的造血干细胞疾病，临床上表现为血小板、红细胞和中性粒细胞过度生成，疾病特异性症状负担重，血管事件发生率高。本综述的重点是批判性地重新评估 MPN 中的血栓形成的临床负担，回顾克隆性造血、JAK2V617F 负荷、炎症和血栓形成之间的临床关联，并为新型原发性和继发性血栓预防策略提供见解。
