摘要
目的:系统评价CALR、JAK2基因突变对原发性骨髓纤维化(PMF)患者临床特征及其预后的影响。方法:计算机检索the Cochrane Library、Pubmed、Embase、中国期刊全文数据库(CNKI)、中文科技期刊数据库(VIP)、中国生物医学文摘(CBM)、万方数据知识服务平台(Wanfang)等中英文数据库,自建库至2018年12月发表的JAK2V617F、CALR基因突变与PMF临床特征及预后的观察性研究。采用Stata15.0软件进行meta分析。结果:共纳入12篇观察性研究,合计2 095例患者,CALR突变组患者533例(25.4%),JAK2型突变患者1 562例(74.6%)。7篇文献报道了血栓形成率,CALR突变PMF患者血栓形成风险低(OR=0.60,95%CI0.37～0.99,P<0.05)。6篇文献报道了急性髓系白血病(AML)转化率,CALR及JAK2基因突变PMF患者合并OR为1.07(95%CI0.69～1.67,P>0.05),未表明两组突变与PMF患者AML转化率风险有关。5篇研究报道了随访期间总生存率的风险比,与CALR基因突变相比,JAK2基因突变组患者总生存率更低(HR=2.77,95%CI1.71～4.47,P<0.05)。结论:CALR基因突变的PMF患者血栓形成风险较JAK2低;CALR突变PMF患者AML转化率较JAK2高,未表明与PMF患者白血病转化风险有关;CALR突变的PMF患者总体生存率比JAK2突变患者高。
Objective:To systematic evaluate the effects of CALR and JAK2 gene mutations on the clinical features and prognosis of patients with primary myelofibrosis(PMF).Method:We retrieved the database such as the Cochrane Library,Pubmed,Embase,China Journal Full-text Database(CNKI),Chinese Science and Technology Periodical Database(VIP),China Biomedical Abstracts(CBM)and Wanfang Data Knowledge Service Platform,in order to research the study of JAK2 V617 Fand CALR gene mutations and clinical features and prognosis of PMF published up to December 2018.Meta-analysis was performed using Stata15.0 software.Result:A total of 12 observational studies were included,with a total of 2095 patients,including 533 patients(25.4%)of CALR gene mutation and 1 562 patients(74.6%)of JAK2-type gene mutation.The thrombosis rate was reported in 7 literatures,and the risk of thrombosis was low in patients with CALR gene mutant PMF(OR=0.60,95%CI 0.37-0.99,P<0.05).Six literatures reported the conversion rate of acute myeloid leukemia(AML),and the ORof PMF patients with CALR and JAK2 gene mutations was 1.07(95%CI0.69-1.67,P>0.05),which did not indicate that the two groups of gene mutations were related to the AML conversion risk of patients with PMF.Five studies reported a hazard ratio of overall survival during follow-up.Compared with the CALR gene mutation,the overall survival rate of the JAK2 gene mutation group was lower(HR=2.77,95%CI 1.71-4.47,P<0.05).Conclusion:The risk of thrombosis in PMF patients with CALR gene mutation is lower than patients with JAK2 gene mutation.The AML conversion rate of patients with CALR mutant PMF is higher than that of JAK2 gene mutation,which shows no correlation with the transformation risk of leukemia in patients with PMF.The overall survival rate of PMF patients with CALR gene mutation is higher than that of patients with JAK2 gene mutation.
作者
宁海玲
张秀莲
NING Hailing;ZHANG Xiulian(Department of Hematology,the First Hospital of Shanxi Medical University,Taiyuan,030001,China)
出处
《临床血液学杂志》
CAS
2019年第3期353-357,共5页
Journal of Clinical Hematology
