中国全科医学2018，Vol.21Issue(12)：1446-1450,5.DOI:10.3969/j.issn.1007-9572.2017.00.240
干扰素α联合沙利度胺治疗JAK2V617F基因突变阳性Bcr/Abl阴性骨髓增殖性肿瘤的临床疗效研究
Therapeutic Effect of Interferonb Alpha Combined with Thalidomide on JAK2V617F Mutation-positive and Bcr/Abl-negative Patients with Myeloproliferative Neoplasm
摘要
Abstract
Objective To investigate the therapeutic effect of interferon alpha(IFN-α) combined with thalidomide in myeloproliferative neoplasm(MPN) patients who are Bcr/Abl-negative and JAK2V617F mutation-positive.Methods According to the inclusion criteria,we enrolled 150 Bcr/Abl-negative MPN hematology outpatients and inpatients treated in Harrison International Peace Hospital from 2006—2016.PCR screening selected 122 patients presenting the JAK2V617F mutation-positive,including 56 polycythemia vera(PV) patients and 66 essential thrombocytosis(ET) patients.According to the tolerance-based therapy,the patients were categorized into 3 groups:group 1[IFN-α plus thalidomide,n=57,including subgroup A1(PV,n=25) and subgroup B1(ET,n=32)],group 2[hydroxyurea plus thalidomide,n=35,including subgroup A2(PV,n=15) and subgroup B2(ET,n=20)],as well as control group[hydroxyurea,n=30,including subgroup A3(PV,n=16) and subgroup B3(ET,n=14)].Clinical remission rate,before treatment,6-month,1-year after treatment JAK2V617F mutation ratio status,morphologic and pathologic features of bone marrow,the disease progression and survival and adverse reaction were recorded.Results There was no significant difference in the clinical remission rate of subgroup A1,subgroup A2 and A3(P>0.05).The clinical remission rate was higher in subgroup B1 compared to B3(P<0.05). Significant interaction and main effects on JAK2V617F mutation were observed between treatment regimen and duration of treatment(P<0.05).In response to 6-month,1-year after treatment,JAK2V617F mutation was reduced in subgroup A1 compared to A2 or A3(P<0.05),and decreased in subgroup B1 compared to B2 or B3(P<0.05).The disease-free survival rates in 3 groups were 91.2%(52/57),80.0%(28/35) and 60.0%(18/30),respectively.Adverse drug reaction rates were low and showed no significant differences between 3 groups.Conclusion The therapeutic responses of JAK2V617F mutation-positive and Bcr/Abl-negative MPN patients to the combination treatment of INF-α and thalidomide can improve the clinical remission rate and disease-free survival rates,and it can reduce JAK2V617F mutation ratio.关键词
骨髓增殖性肿瘤/干扰素α/沙利度胺/Janus激酶2/突变Key words
Myeloproliferative neoplasm/Interferon-α/Thalidomide/Janus kinase 2/Mutation分类
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王冬梅,王洪芬,李英华,刘珊,李志赏,石锐..干扰素α联合沙利度胺治疗JAK2V617F基因突变阳性Bcr/Abl阴性骨髓增殖性肿瘤的临床疗效研究[J].中国全科医学,2018,21(12):1446-1450,5.基金项目
2013年衡水市科学技术研究与发展计划(13002A) （13002A）
