干扰素α联合沙利度胺对Bcr/Abl阴性骨髓增殖性肿瘤JAK2V617F基因突变的影响  

Clinical research on effect of interferon alpha and thalidomide on JAK2V617F gene expression in patients with Bcr / Abl negative myeloproliferative neoplasm

作　　者：王冬梅[1] 王洪芬[1] 李英华[1] 符敬坦 刘珊[1] 李志赏[1] 石锐[1] 

机构地区：[1]哈励逊国际和平医院血液科,河北省衡水市053000 [2]河北省衡水市妇幼保健院,053000

出　　处：《实用医学杂志》2015年第10期1607-1610,共4页The Journal of Practical Medicine

摘　　要：目的：研究干扰素α联合沙利度胺在治疗Bcr/Abl阴性骨髓增殖性肿瘤（MPN）中的有效性、安全性及对JAK2V617F基因表达的影响。方法：分析44例应用干扰素α联合沙利度胺治疗超过6个月的Bcr/Abl阴性骨髓增殖性肿瘤患者的临床资料及JAK2V617F突变比例,并与同期应用羟基脲联合沙利度胺治疗的27例患者、单用羟基脲治疗的21例患者作为对照,治疗前后应用实时荧光定量PCR方法检测JAK2V617F突变比例。结果 ：44例干扰素α联合沙利度胺治疗组中的真性红细胞增多症及原发性血小板增多症分别有89%、92%达到完全或部分缓解,与羟基脲联合沙利度胺及单用羟基脲两组比较,差异均有显著性（P均〈0.01）,随访0.5~8年,44例干扰素α联合沙利度胺治疗组的真性红细胞增多症、原发性血小板增多症JAK2V617F突变比例分别下降了53.76±18.43及30.14±22.75,其中5例患者JAK2V617F突变转阴,持续15~24个月。而羟基脲联合沙利度胺组及单用羟基脲组突变比例无明显下降,无突变转阴者。干扰素α及沙利度胺治疗过程中的副作用分别为流感样症状及便秘、周围神经炎,但均能耐受。结论：干扰素α联合沙利度胺治疗Bcr/Abl阴性MPN能取得很好的疗效及较好的耐受性,JAK2V617F或可作为骨髓增殖性肿瘤患者能否达到分子生物学缓解的主要标志之一。Objective To observe the efficacy, safety and effect on JAK2V617F gene expression of interferon-a and thalidomide in the treatment of Bcr/Abl negative myeloproliferative neoplasms （MPN）. Methods Clinical data and JAK2V617F mutation rate of 44 patients with Bcr/Abl negative MPN were analyzed. The patients were treated with interferon-a and thalidomide more than 6 months. 27 patients treated with hydroxyurea and thalidomide, and 21 patients treated with hydroxyurea. The rate of JAK2V617F mutation was measured by real-time PCR before and after treatment. Results 89% of polycythemia vera patients and 92% of essential thrombocythemia patients in the 44 patients treated with interferon-α and thalidomide reached the completed remission or partial remission, the result of which is significant compared with the control group treated with hydroxyurea and thalidomide and with hydroxyurea （P 〈 0.01）. 0.5 to 8 years fellowship on patients treated with interferon-a and thalidomide revealed JAK2V617F mutation rate decreased 53.76±18.43 in polycythemia vera patients and 30.14±22.75 in essential thrombocythemia patients. JAK2V617F mutation turned to negative in 5 of these patients, lasting for 15 to 24 months. While the mutation rate in control groups has no obvious decrease, nobody ＇s records turned to negative. The side effects of interferon-a and thalidomide were influenza-like symptoms, constipation and peripheral neuritis, which can all be tolerable. Conclusions The therapeutic response of interferon-a and thalidomide in treatment of Bcr/Abl negativeMPN were significant and tolerable. JAK2V617F could be used as one of the important markers to evaluate whether MPN patients have achieved molecular biologic remission.

关 键 词：骨髓增殖性肿瘤 干扰素Α 沙利度胺 JAK2V617F 

分 类 号：R733.3[医药卫生—肿瘤]