BCR-ABL融合基因阴性的骨髓增殖性肿瘤血栓形成相关因素及治疗的研究进展  

Research Progress of Thrombogenesis Related Factors and Therapy of BCR-ABL Negative Myeloproliferative Neoplasms

作　　者：许鑫 赵越[1] 高玉娟[1] 苏雁华[1] XU Xin;ZHAO Yue;GAO Yujuan;SU Yanhua(Department of Hematology,the First Affiliated Hospital of Harbin Medical University,Harbin 150001,China)

机构地区：[1]哈尔滨医科大学附属第一医院血液内科,哈尔滨150001

出　　处：《医学综述》2021年第18期3634-3640,共7页Medical Recapitulate

摘　　要：断裂点簇集区-艾贝尔逊白血病病毒(BCR-ABL)融合基因阴性的骨髓增殖性肿瘤(MPNs)具有血栓形成的风险,是导致患者高致残率和高病死率的重要原因。虽然近年来关于BCR-ABL融合基因阴性的MPNs患者血栓形成相关因素及治疗的研究较多,但其发病机制目前尚未完全明确。基因突变、血细胞相关参数、凝血相关因素、生化指标以及某些特殊的临床表现等均可能是BCR-ABL融合基因阴性的MPNs患者血栓形成的相关因素。放血疗法、抗血小板治疗、细胞减少性治疗、Janus激酶2抑制剂及抗凝治疗等是BCR-ABL融合基因阴性的MPNs最常用的治疗方法。未来希望通过对BCR-ABL融合基因阴性的MPNs患者血栓形成相关因素及治疗研究的深入,可进一步降低BCR-ABL融合基因阴性的MPNs患者的血栓发生率。Breakpoint cluster region-abelson leukemia virus(BCR-ABL)fusion gene negative myeloproliferative neoplasms(MPNs)are prone to thrombosis which is an important cause of high disability and mortality.Although there are many studies on the factors of thrombosis and treatment for the patients with BCR-ABL negative MPNs in recent years,the pathogenesis is not yet fully and clearly understood.Gene mutation,blood cell related parameters,coagulation related factors,biochemical indicators and some special clinical manifestations may be related factors for thrombosis in patients with BCR-ABL negative MPNs.Bloodletting therapy,antiplatelet therapy,cytoreductive therapy,Janus kinase inhibitors and anticoagulant therapy are the most commonly used treatments for BCR-ABL negative MPNs.In the future,further researches on the associated factors and therapy of thrombosis in BCR-ABL negative MPNs are expected to help to further reduce the incidence of thrombosis in the patients.

关 键 词：骨髓增殖性肿瘤 血栓 断裂点簇集区-艾贝尔逊白血病病毒融合基因 基因突变 血细胞计数 

分 类 号：R733.3[医药卫生—肿瘤]