中华内科杂志
中華內科雜誌
중화내과잡지
CHINESE JOURNAL OF INTERNAL MEDICINE
2013年
7期
585-589
,共5页
孙莺心%朱明清%何广胜%王秀丽%方宝枝%路丛%刘真真%吴倩%杨永
孫鶯心%硃明清%何廣勝%王秀麗%方寶枝%路叢%劉真真%吳倩%楊永
손앵심%주명청%하엄성%왕수려%방보지%로총%류진진%오천%양영
贫血,再生障碍性%血红蛋白尿,阵发性%免疫抑制法%环孢菌素%抗人胸腺细胞免疫球蛋白
貧血,再生障礙性%血紅蛋白尿,陣髮性%免疫抑製法%環孢菌素%抗人胸腺細胞免疫毬蛋白
빈혈,재생장애성%혈홍단백뇨,진발성%면역억제법%배포균소%항인흉선세포면역구단백
Anemia,aplastic%Hemoglobinuria,paroxysmal%Immunosuppression%Cyclosporine%Anti-thymocyte immunoglobulin
目的 探索再生障碍性贫血(AA)免疫抑制治疗(IST)后阵发性睡眠性血红蛋白尿症(PNH)克隆变化情况及其临床意义.方法 将2008年1月至2012年2月收治的186例AA患者纳入本研究,其中55例重型AA(SAA)予抗胸腺细胞球蛋白(ATG)联合环孢素A(CsA)治疗,131例非重型AA(NSAA)予CsA治疗.治疗前所有患者进行PNH克隆筛查,治疗后进行随访.中位随访时间22(18 ～76)个月.结果 SAA组10例(18.9％)患者出现PNH克隆,NSAA组9例(7.4％)出现PNH克隆,前者出现PNH克隆比例高于后者(t=5.041,P=0.025),治疗后SAA组在6、12、18、＞24个月时PNH克隆规模大于NSAA组(13.38％比5.67％、14.88％比5.31％、20.00％比5.47％、18.85％比9.08％,P值均＜0.05).PNH克隆对2种IST方案疗效影响的差异无统计学意义.结论 AA患者予ATG联合CsA或CsA治疗后,均可出现PNH克隆,SAA患者ATG治疗后更明显.IST前后伴PNH克隆不影响IST疗效.
目的 探索再生障礙性貧血(AA)免疫抑製治療(IST)後陣髮性睡眠性血紅蛋白尿癥(PNH)剋隆變化情況及其臨床意義.方法 將2008年1月至2012年2月收治的186例AA患者納入本研究,其中55例重型AA(SAA)予抗胸腺細胞毬蛋白(ATG)聯閤環孢素A(CsA)治療,131例非重型AA(NSAA)予CsA治療.治療前所有患者進行PNH剋隆篩查,治療後進行隨訪.中位隨訪時間22(18 ～76)箇月.結果 SAA組10例(18.9％)患者齣現PNH剋隆,NSAA組9例(7.4％)齣現PNH剋隆,前者齣現PNH剋隆比例高于後者(t=5.041,P=0.025),治療後SAA組在6、12、18、＞24箇月時PNH剋隆規模大于NSAA組(13.38％比5.67％、14.88％比5.31％、20.00％比5.47％、18.85％比9.08％,P值均＜0.05).PNH剋隆對2種IST方案療效影響的差異無統計學意義.結論 AA患者予ATG聯閤CsA或CsA治療後,均可齣現PNH剋隆,SAA患者ATG治療後更明顯.IST前後伴PNH剋隆不影響IST療效.
목적 탐색재생장애성빈혈(AA)면역억제치료(IST)후진발성수면성혈홍단백뇨증(PNH)극륭변화정황급기림상의의.방법 장2008년1월지2012년2월수치적186례AA환자납입본연구,기중55례중형AA(SAA)여항흉선세포구단백(ATG)연합배포소A(CsA)치료,131례비중형AA(NSAA)여CsA치료.치료전소유환자진행PNH극륭사사,치료후진행수방.중위수방시간22(18 ～76)개월.결과 SAA조10례(18.9％)환자출현PNH극륭,NSAA조9례(7.4％)출현PNH극륭,전자출현PNH극륭비례고우후자(t=5.041,P=0.025),치료후SAA조재6、12、18、＞24개월시PNH극륭규모대우NSAA조(13.38％비5.67％、14.88％비5.31％、20.00％비5.47％、18.85％비9.08％,P치균＜0.05).PNH극륭대2충IST방안료효영향적차이무통계학의의.결론 AA환자여ATG연합CsA혹CsA치료후,균가출현PNH극륭,SAA환자ATG치료후경명현.IST전후반PNH극륭불영향IST료효.
Objective To evaluate the evolution of paroxysmal nocturnal hemoglobinuria (PNH) clone and its clinical significance before and after immunosuppressive therapy (IST) in patients with aplastic anemia (AA).Methods A total of 186 patients diagnosed as AA were enrolled in this study.Among them,55 patients were diagnosed as severe AA (SAA) and treated with cyclosporine (CsA) plus antithymocyteglobulin (ATG),131 were diagnosed as non SAA (NSAA) and treated with CsA alone.All patients were screened for PNH clone by flow cytometry before treatment and followed up for 18-76 months,with a median time of 22 months.Results Positive PNH clones were detected in 10 SAA (18.9％) patients,significantly more than that of NSAA group [9 patients (7.4％),t =5.041,P =0.025].The proportions of PNH clones in SAA group at 6,12,24 and ＞ 24 months were 13.38％,14.88％,20.00％ and 18.85％,respectively,also significantly higher than those of NSAA patients (5.67％,5.31％,5.47％ and 9.08％,all P values ＜0.05).Clinical response rates were comparable in both ATG + CsA or CsA alone groups no matter PNH clone was positive or negative.Conclusions PNH clone are detectable in AA patients either treated with ATG plus CsA or CsA alone,and more significant by ATG plus CsA.Whether PNH clone occurres before or after IST does not affect the therapeutic efficacy.
