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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。
This study investigated the efficacy and safety of interferon-alpha-2b (IFN-α-2b) in the treatment of polycythemia vera (PV) and post-PV MF. IFN-α-2b was used in 30 patients with JAK2V617F mutation-positive PV. The changes of JAK2V617F mutation load before and after treatment with IFN-α-2b were detected by real-time quantitative PCR, and the correlation with the clinical indexes was also studied. The results showed that the median follow-up time of 29 evaluable patients was 24 (12-42) months. Hematologic complete remissions occurred in 10%, 48%, 72% and 78% of patients at 6, 12, 24 and 36 months after IFN-a-2b treatment, respectively. Monitoring changes in the JAK2V617F mutation load showed that molecular remission rates were 41%, 76%, 89%, and 89% at 6, 12, 24 and 36 months after IFNα2b treatment, respectively. 4 patients after IFN-α-2b treatment JAK2V617F mutation gene negative, discontinuation of 6-12 months without recurrence. The reduction of JAK2V617F mutation load in 53 of 18 patients with post-PV MF treated with IFN-α-2b was significantly different from that of non-myelinated patients (32 ± 22%) (P < p = 0.031). Conclusion: IFN-α-2b can selectively act on PV malignant clones, reduce the load of JAK2V617F mutation and make the molecular level of PV patients relieve, especially for post-PV MF.