干扰素α-2b治疗真性红细胞增多症及其继发骨髓纤维化患者分子生物学反应的研究  

Interferon-alpha-2b Induces Molecular Responses of Patients with Polycythemia Vera and Its Post-polycythemic Myelofibrosis

作　　者：白洁 薛艳萍 张磊[1] 李庆华 范冬梅 李彬 徐世才 李大鹏 孙秀娟 江波 杨仁池 

机构地区：[1]中国医学科学院、北京协和医学院血液学研究所、血液病医院,国家实验血液学重点实验室,天津300020

出　　处：《中国实验血液学杂志》2011年第2期444-449,共6页Journal of Experimental Hematology

摘　　要：本研究探讨干扰素-alpha-2b(IFN-α-2b)治疗真性红细胞增多症(polycythemia vera,PV)及PV后骨髓纤维化(post-PV MF)患者的有效性和安全性。对30例伴有JAK2V617F突变阳性的PV患者应用IFN-α-2b治疗,通过实时定量PCR的方法检测IFN-α-2b治疗前后JAK2V617F突变负荷的变化,并研究其与临床指标的相关性。结果表明,29例可评价的患者中位随访时间为24(12-42)个月。在IFN-α-2b治疗后6、12、24及36个月分别有10%、48%、72%及78%患者发生血液学完全缓解。监测JAK2V617F突变负荷的变化显示,IFNα2b治疗后6、12、24及36个月发生分子水平缓解率分别为41%、76%、89%及89%。4例患者经IFN-α-2b治疗后JAK2V617F突变基因转阴,停药6-12个月未复发。7例post-PV MF患者应用IFN-α-2b治疗后12月JAK2V617F突变负荷减低程度[(53±18)%]较非骨髓纤维化PV患者[(32±22)%]有显著性差异(p=0.031)。结论:IFN-α-2b可选择作用于PV恶性克隆,降低JAK2V617F突变负荷,使PV患者发生分子水平缓解,尤其对post-PV MF的作用较明显。To evaluate the efficacy and safety of interferon-alpha-2b（IFN-α-2b） in polycythemia vera patients（PV patient） with or without post-polycythemic myelofibrosis（post-PV MF）, 30 patietns with mutanted JAK2V617F were enrolled in this study,from which 29 patients were evaluable.The percentage of mutanted JAK2V617F allele（V617F%） was evaluated by real-time polymerase chain reaction（RT-PCR） before and after treatment with IFN-α-2b.The correlation of V617F allele burden with the major clinical outcomes was studied.Adverse effects appeared in patients was observed.The results showed that the median follow-up was 24（12-42） months for 29 evaluable patients.Complete hematologic response was achieved in 10%,48%,72% and 78% of patients after treatment for 6,12,24 and 36 months respectively.The detection of V617F allele burden revealed that the molecular remission of patients（V617F%） was achieved in 41%,76%,89% and 89% after treatment for 6,12,24 and 36 months respectively.Molecular complete remission（JAK2V617F undetectable） was achieved in 4 patients,lasted from 6 to 12 months after IFN-α-2b discontinuation.The decrease of V617F% in patients with post-PV MF was significantly higher than that in patients without post-PV MF（53±18% vs 32±22%,respectively;p=0.031）after treatment for 12 months.PV patients had a good tolerance to IFN-α-2b.It is concluded that IFN-α-2b can decrease the mutated V617F allele burden.Patients with PV,especially with post-PV MF,can achieve molecular remission after treatment with IFN-α-2b.

关 键 词：真性红细胞增多症 骨髓纤维化 JAK2V617F突变 干扰素-Α-2B 

分 类 号：R555.1[医药卫生—血液循环系统疾病]