干扰素-α对骨髓增殖性肿瘤JAK2V617F基因表达的影响

【目的】 研究干扰素-α在治疗骨髓增殖性肿瘤(MPN)中的疗效及对JAK2V617F基因表达的影响。【方法】 分析73例应用干扰素-α治疗超过6个月的MPN患者的临床资料及JAK2V617F基因表达,并与同期单用羟基脲治疗的20例MPN做对照。治疗前后采用AS-PCR方法检测JAK2V617F基因表达。【结果】 干扰素-α治疗组中真性红细胞增多症、原发性血小板增多症分别有85.2 %、87 %达到完全或部分缓解(与对照组单用羟基脲治疗比较,P < 0.01)。随访1 ~ 6年,56例干扰素-α治疗组的真性红细胞增多症、原发性血小板增多症JAK2V617F突变基因转阴率分别为54%、41%,转阴患者100%达到临床及血液学完全缓解,而对照组无一例转阴。干扰素-α治疗过程中的副作用主要表现为流感样症状,但均能耐受。【结论】干扰素-α治疗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。

Effect of Interferon Alpha on JAK2V617F Gene Expression in Patients with Myeloproliferative Neoplasm

【Objective】 To explore the therapeutic effect of interferon alpha (IFN-α) in patients with myeloproliferative neoplasm (MPN) as well as its effect on JAK2V617F gene expression. 【Methods】 In the present study, clinical data and JAK2V617F gene expression were evaluated in 73 cases of MPN patients who had been treated with IFN-α for more than 6 months, in comparison to the control group which consisted of 20 cases of MPN patients treated with hydroxyurea. The expression of JAK2V617F was evaluated before and after the treatment by AS-PCR method.【Results】After 6 months of IFN-α treatment,85.2% of MPN patients with polycythemia vera and 87% of MPN patients with essential thrombocythemia achieved complete remission or partial remission. The difference is statistically significant in comparison to the results of the control group with hydroxyurea treatment(P < 0.01). In MPN patients presented with positive JAK2V617F gene expression, continuous treatment with IFN-α1 for 1 to 6 years reversed the gene expression to negative in 54% (13/24) of the cases with polycythemia vera and 41% (13/32) of the cases with essential thrombocythemia. All of these cases with reversed gene expression remained complete remission. No reversion of JAK2V617F gene expression could be observed in the control group with hydroxyurea treatment. Although the major side effect of IFN-α was flu-like syndrome, all of the patients in this study could tolerate the therapy well. 【Conclusion】 In the present study, IFN-α has shown promising therapeutic response and patient tolerance in the management of MPN. JAK2V617F might be used as one of the important markers to evaluate whether MPN patients have been achieved molecular biologic remission.