聚乙二醇干扰素-α治疗JAK2V617F基因突变阳性骨髓增殖性肿瘤患者的疗效及安全性评价  

Efficacy and safety of pegylated interferon-α on myeloproliferative neoplasm patients with JAK2V617F gene mutation

作　　者：丁莉 邢宏运 韩丽英 李晓明 DING Li;XING Hongyun;HAN Liying;LI Xiaoming(Department of Hematology Medicine,APfiliated Hospital of Southwest Medical University,Luzhou 646000,Sichuan Province,China)

机构地区：[1]西南医科大学附属医院血液科,四川泸州646000

出　　处：《肿瘤》2018年第8期792-798,共7页Tumor

摘　　要：目的 :评价长效干扰素(interferon,INF)聚乙二醇-INF-α(pegylated IFN-α,PEG-IFN-α)治疗JAK 2V 617F基因突变阳性慢性骨髓增殖性肿瘤(myeloproliferative neoplasm,MPN)患者的临床疗效及安全性。方法:回顾性分析了85例分别接受6个月以上PEG-IFN-α(38例)(PEG-IFN-α组)或IFN-α(47例)(IFN-α组)治疗的JAK 2V 617F基因突变阳性MPN患者[45例为真性红细胞增多症(polycythemia vera,PV),40例为原发性血小板增多症(essential thrombocytosis,ET)]的临床资料,并比较2种IFN的临床疗效及不良反应。结果:PEG-IFN-α组PV患者及ET患者的完全缓解率和总缓解率分别为60.0%、90.0%和61.1%、88.9%,均明显高于IFN-α组的40.0%、72.0%和40.9%、63.6%(P值均<0.05);PEG-IFN-α组PV和ET患者的5年无进展生存率分别为85.0%和94.4%,明显高于IFN-α组的68.0%和72.7%,差异有统计学意义(P值均<0.05)。PEG-IFN-α较IFN-α在改善PV及ET患者血管舒缩功能障碍相关症状如瘙痒、红斑性肢痛及肢端感觉异常方面具有明显优势(P值均<0.05)。此外,PEG-IFN-α组PV及ET患者的血液学(1～2级)及非血液学(流感样症状1～2级)不良反应较IFN-α组PV及ET患者明显更多(P值均<0.05),但减药、停药或对症处理后多数患者可耐受。结论:长效干扰素PEG-IFN-α可能是JAK 2V 617F基因突变阳性的ET和PV患者的有效治疗用药,可使其获得较好临床缓解及无进展生存。Objective： To evaluate the efficacy and safety of pegylated interferonalpha（PEG-IFN-α） in the treatment of myeloproliferative neoplasm（MPN） patients with JAK2 V617 F gene mutation. Methods： A retrospective analysis was performed on the 85 cases of MPN patients with JAK2 V617 F gene mutation, which included 45 polycythemia vera（PV） patients and 40 essential thrombocytosis（ET） patients. These patients randomly received the treatment of PEG-IFN-α（38 cases） and IFN-α（47 cases） for more than 6 months, respectively. Then the clinical treatment outcome and adverse effects of PEG-IFN-α and IFN-α were compared.Results： The complete remission rate and overall remission rate of PV patients received PEG-IFN-αtreatment were 60.0% and 90.0%, those of ET patients were 61.1% and 88.9%, which were significantly higher than those of IFN-α group（40.0% and 72.0%; 40.9% and 63.6%）（all P〈0.05）. The 5-year progression-free survival rate of PEG-IFN-α-treated patients were 85.0% and 94.4%, significantly higher than those of IFN-α-treated counterparts（68.0% and 72.7%）（all P〈0.05）. In addition, PEG-IFN-αhad significant advantages in improving patients＇ vasomotor dysfunction-related symptoms such as pruritus, erythromelalgia, and distal paresthesias as compared with IFN-α（all P〈0.05）. Furthermore, compared with IFN-α group, there were higher risks of hematologic adverse reactions（grade 1-2） and non-hematologic adverse reactions（flu-like symptoms, grade 1-2） in PEG-IFN-α-treated group（all P〈0.05）, but the reducing and stopping drugs treatment or the symptomatic treatment could relieve these adverse reactions for the most PEG-IFN-α-treated patients.Conclusion： PEG-IFN-α may be a promising therapy for the PV and ET patients with JAK2 V617 Fgene mutation, which has favorable hematologic response and progression-free survival rate.

关 键 词：骨髓增殖性肿瘤 JAK2V617F基因突变 干扰素Α 治疗结果 药物相关性副作用和不良反应 

分 类 号：R737.9[医药卫生—肿瘤]