中华血液学杂志
中華血液學雜誌
중화혈액학잡지
Chinese Journal of Hematology
2015年
11期
942-946
,共5页
王静雅%秦铁军%徐泽锋%张悦%张宏丽%方力维%潘丽娟%胡耐博%曲士强
王靜雅%秦鐵軍%徐澤鋒%張悅%張宏麗%方力維%潘麗娟%鬍耐博%麯士彊
왕정아%진철군%서택봉%장열%장굉려%방력유%반려연%호내박%곡사강
骨髓增生异常综合征%环孢素%沙利度胺%cereblon基因
骨髓增生異常綜閤徵%環孢素%沙利度胺%cereblon基因
골수증생이상종합정%배포소%사리도알%cereblon기인
Myelodysplastic syndromes%Cyclosporine A%Thalidomide%Cereblon
目的 评价环孢素(CsA)联合沙利度胺治疗国际预后积分系统(IPSS)低危/中危-1骨髓增生异常综合征(MDS)患者的远期疗效及预后影响因素.方法 回顾性分析CsA联合沙利度胺治疗的76例IPSS低危/中危-1 MDS患者临床资料.采用PCR联合直接测序法检测患者cereblon基因rs 1672753位点基因型.结果 76例患者中,男48例,女28例,中位年龄41(18～70)岁.CsA联合沙利度胺治疗后,40例(53％)获得血液学改善(HI),其中红系反应(HI-E)率为43％(73例中31例),中性粒细胞反应(HI-N)率为30％(50例中15例),血小板反应(HI-P)率为31％(58例中18例).59例红细胞输注依赖患者中27例(46％)获得HI-E并脱离输血.HI中位维持时间为22(1～131+)个月.单因素分析显示骨髓原始细胞≤2％的患者疗效持续时间更长(P=0.010).cereblon基因rs1672753位点基因型与HI率及治疗反应的维持时间均无明显相关性(P值均＞0.05).67例未行造血干细胞移植患者中位生存时间为82(95％CI38～126)个月.多因素分析显示IPSS-R分组(HR=3.461,95％CI 1.126～10.639,p=0.030)、年龄≥60岁(HR=4.120,95％CI1.070～15.867,P=0.040)以及HI-N (HR=7.733,95％CI 1.007～59.396,P=0.049)为影响患者生存时间的独立预后因素.结论 CsA联合沙利度胺治疗能长期改善IPSS低危/中危-1 MDS患者贫血症状,不良反应轻.在此组患者中未能验证cereblon基因rs1672753位点基因型对疗效的预测价值.
目的 評價環孢素(CsA)聯閤沙利度胺治療國際預後積分繫統(IPSS)低危/中危-1骨髓增生異常綜閤徵(MDS)患者的遠期療效及預後影響因素.方法 迴顧性分析CsA聯閤沙利度胺治療的76例IPSS低危/中危-1 MDS患者臨床資料.採用PCR聯閤直接測序法檢測患者cereblon基因rs 1672753位點基因型.結果 76例患者中,男48例,女28例,中位年齡41(18～70)歲.CsA聯閤沙利度胺治療後,40例(53％)穫得血液學改善(HI),其中紅繫反應(HI-E)率為43％(73例中31例),中性粒細胞反應(HI-N)率為30％(50例中15例),血小闆反應(HI-P)率為31％(58例中18例).59例紅細胞輸註依賴患者中27例(46％)穫得HI-E併脫離輸血.HI中位維持時間為22(1～131+)箇月.單因素分析顯示骨髓原始細胞≤2％的患者療效持續時間更長(P=0.010).cereblon基因rs1672753位點基因型與HI率及治療反應的維持時間均無明顯相關性(P值均＞0.05).67例未行造血榦細胞移植患者中位生存時間為82(95％CI38～126)箇月.多因素分析顯示IPSS-R分組(HR=3.461,95％CI 1.126～10.639,p=0.030)、年齡≥60歲(HR=4.120,95％CI1.070～15.867,P=0.040)以及HI-N (HR=7.733,95％CI 1.007～59.396,P=0.049)為影響患者生存時間的獨立預後因素.結論 CsA聯閤沙利度胺治療能長期改善IPSS低危/中危-1 MDS患者貧血癥狀,不良反應輕.在此組患者中未能驗證cereblon基因rs1672753位點基因型對療效的預測價值.
목적 평개배포소(CsA)연합사리도알치료국제예후적분계통(IPSS)저위/중위-1골수증생이상종합정(MDS)환자적원기료효급예후영향인소.방법 회고성분석CsA연합사리도알치료적76례IPSS저위/중위-1 MDS환자림상자료.채용PCR연합직접측서법검측환자cereblon기인rs 1672753위점기인형.결과 76례환자중,남48례,녀28례,중위년령41(18～70)세.CsA연합사리도알치료후,40례(53％)획득혈액학개선(HI),기중홍계반응(HI-E)솔위43％(73례중31례),중성립세포반응(HI-N)솔위30％(50례중15례),혈소판반응(HI-P)솔위31％(58례중18례).59례홍세포수주의뢰환자중27례(46％)획득HI-E병탈리수혈.HI중위유지시간위22(1～131+)개월.단인소분석현시골수원시세포≤2％적환자료효지속시간경장(P=0.010).cereblon기인rs1672753위점기인형여HI솔급치료반응적유지시간균무명현상관성(P치균＞0.05).67례미행조혈간세포이식환자중위생존시간위82(95％CI38～126)개월.다인소분석현시IPSS-R분조(HR=3.461,95％CI 1.126～10.639,p=0.030)、년령≥60세(HR=4.120,95％CI1.070～15.867,P=0.040)이급HI-N (HR=7.733,95％CI 1.007～59.396,P=0.049)위영향환자생존시간적독립예후인소.결론 CsA연합사리도알치료능장기개선IPSS저위/중위-1 MDS환자빈혈증상,불량반응경.재차조환자중미능험증cereblon기인rs1672753위점기인형대료효적예측개치.
Objective To investigate the long-term outcome of cyclosporin A (CsA) combined with thalidomide regime for Chinese patients with IPSS low/intermediate-1 myelodysplastic syndromes (MDS) without del (5q) and the predictive variables which could impact the response to the therapy.Methods Seventy-six MDS patients who were treated with these drugs at a single institute in China were retrospectively analyzed.The polymorphism of cerebion gene,rs1672753,was detected in patients of this cohort by PCR and direct sequencing.Results A total of 53％ of patients showed hematological improvement (HI) to the therapy.Thirty-one patients (31/73,43％) achieved erythrocyte response (HI-E);15 patients (15/50,30％) achieved neutrophil response (HI-N);18 patients (18/58,31％) achieved platelet response (HI-P).Twenty-seven of the 50 patients (46％) who were dependent on red blood cell transfusion achieved HI-E and became independent of transfusion.The median duration of response among the responders was 22 months (range,1-131 + months).Bone marrow blasts ≤2％ was the only factor associated with longer response duration in univariate analysis (P=0.010).There was no significant difference between the two groups of celeblon gene rs 1672753 polymorphism either on the response rate or the response duration.The median survival of 67 patients without stem cell transplantation was 82 months.In multivariate analyses,factors significantly correlated with survival were IPSS-R (HR=3.461,95％CI 1.126-10.639,P=0.030),age≥60 y (HR=4.120,95％CI 1.070-15.867,P=0.040) and HI-N (HR=7.733,95％CI 1.007-59.396,P=0.049).Conclusion CsA combined with thalidomide regime could improve the anemia symptom in low/int-1 risk MDS patients without del (5q).The predictive value of cereblon gene polymorphism,rs1672753,could not be verified in this study.
