中华医学杂志(英文版)
中華醫學雜誌(英文版)
중화의학잡지(영문판)
CHINESE MEDICAL JOURNAL
2002年
3期
398-401
,共4页
赵明峰%邵宗鸿%李克%陈桂彬%刘鸿%张益枝%和虹%施均%何广胜%储榆林%杨天楹
趙明峰%邵宗鴻%李剋%陳桂彬%劉鴻%張益枝%和虹%施均%何廣勝%儲榆林%楊天楹
조명봉%소종홍%리극%진계빈%류홍%장익지%화홍%시균%하엄성%저유림%양천영
阵发性睡眠性血红蛋白尿症%临床特征%诊断%治疗
陣髮性睡眠性血紅蛋白尿癥%臨床特徵%診斷%治療
진발성수면성혈홍단백뇨증%림상특정%진단%치료
paroxysmal nocturnal hemoglobinuria%clinical features%diagnosis%treatment
目的 研究九十年代阵发性睡眠性血红蛋白尿症（PNH）患者的临床特征,以增加对PNH新的认识,指导PNH的诊断及治疗.方法 对1990年1月至1999年11月我院诊断的78例新发PNH患者的临床表现、实验室检查及治疗情况进行了回顾性分析.结果 ①与八十年代的病例相比,新发PNH患者的发病年龄有所增大（中位年龄由27岁升至34岁）；女性病例增多（由18.5%升至38.5%）；无血红蛋白尿发作病例增多（由24.2%升至38.5%）；血栓形成发生率仍较低（3.0%对6.4%）. ②所有患者均无家族遗传史. ③部分患者表现类似骨髓增生异常综合征：骨髓有2-3系病态造血（19.2%）,染色体核型异常（12.2%）,姊妹染色单体分染阴性（8.9%）；部分患者表现类似再生障碍性贫血：骨髓增生减低（12.3%）,Ham's试验阴性（34.2%）.但100.0%（25/25例）患者外周血红细胞及粒细胞CD55或CD59表达异常,可作为鉴别诊断的特异性指标.④83.8%患者对肾上腺糖皮质激素为主的治疗有效,但易复发,1年复发率为54.2%.8例对肾上腺糖皮质激素治疗效果差或有依赖的难治性、复发性PNH用低剂量化疗（MP方案：马法兰2-6?mg*d-1；强的松0.5?mg*kg-1*d-1）治疗有效率为62.5%,而且均未出现严重的骨髓抑制及其它毒副反应.结论 PNH作为一种后天获得性疾病,更多见于成年男性.CD55、CD59的检测有助于提高PNH的检出率,大部分的PNH患者对肾上腺糖皮质激素为主的治疗反应良好,对难治性、复发性PNH患者可试用低剂量化疗.
目的 研究九十年代陣髮性睡眠性血紅蛋白尿癥（PNH）患者的臨床特徵,以增加對PNH新的認識,指導PNH的診斷及治療.方法 對1990年1月至1999年11月我院診斷的78例新髮PNH患者的臨床錶現、實驗室檢查及治療情況進行瞭迴顧性分析.結果 ①與八十年代的病例相比,新髮PNH患者的髮病年齡有所增大（中位年齡由27歲升至34歲）；女性病例增多（由18.5%升至38.5%）；無血紅蛋白尿髮作病例增多（由24.2%升至38.5%）；血栓形成髮生率仍較低（3.0%對6.4%）. ②所有患者均無傢族遺傳史. ③部分患者錶現類似骨髓增生異常綜閤徵：骨髓有2-3繫病態造血（19.2%）,染色體覈型異常（12.2%）,姊妹染色單體分染陰性（8.9%）；部分患者錶現類似再生障礙性貧血：骨髓增生減低（12.3%）,Ham's試驗陰性（34.2%）.但100.0%（25/25例）患者外週血紅細胞及粒細胞CD55或CD59錶達異常,可作為鑒彆診斷的特異性指標.④83.8%患者對腎上腺糖皮質激素為主的治療有效,但易複髮,1年複髮率為54.2%.8例對腎上腺糖皮質激素治療效果差或有依賴的難治性、複髮性PNH用低劑量化療（MP方案：馬法蘭2-6?mg*d-1；彊的鬆0.5?mg*kg-1*d-1）治療有效率為62.5%,而且均未齣現嚴重的骨髓抑製及其它毒副反應.結論 PNH作為一種後天穫得性疾病,更多見于成年男性.CD55、CD59的檢測有助于提高PNH的檢齣率,大部分的PNH患者對腎上腺糖皮質激素為主的治療反應良好,對難治性、複髮性PNH患者可試用低劑量化療.
목적 연구구십년대진발성수면성혈홍단백뇨증（PNH）환자적림상특정,이증가대PNH신적인식,지도PNH적진단급치료.방법 대1990년1월지1999년11월아원진단적78례신발PNH환자적림상표현、실험실검사급치료정황진행료회고성분석.결과 ①여팔십년대적병례상비,신발PNH환자적발병년령유소증대（중위년령유27세승지34세）；녀성병례증다（유18.5%승지38.5%）；무혈홍단백뇨발작병례증다（유24.2%승지38.5%）；혈전형성발생솔잉교저（3.0%대6.4%）. ②소유환자균무가족유전사. ③부분환자표현유사골수증생이상종합정：골수유2-3계병태조혈（19.2%）,염색체핵형이상（12.2%）,자매염색단체분염음성（8.9%）；부분환자표현유사재생장애성빈혈：골수증생감저（12.3%）,Ham's시험음성（34.2%）.단100.0%（25/25례）환자외주혈홍세포급립세포CD55혹CD59표체이상,가작위감별진단적특이성지표.④83.8%환자대신상선당피질격소위주적치료유효,단역복발,1년복발솔위54.2%.8례대신상선당피질격소치료효과차혹유의뢰적난치성、복발성PNH용저제양화료（MP방안：마법란2-6?mg*d-1；강적송0.5?mg*kg-1*d-1）치료유효솔위62.5%,이차균미출현엄중적골수억제급기타독부반응.결론 PNH작위일충후천획득성질병,경다견우성년남성.CD55、CD59적검측유조우제고PNH적검출솔,대부분적PNH환자대신상선당피질격소위주적치료반응량호,대난치성、복발성PNH환자가시용저제양화료.
Objective To learn more about the clinical and laboratory features of patients with paroxysmal nocturnal hemoglobinuria (PNH) diagnosed in the past ten years. Methods Clinical and laboratory data for 78 cases of PNH diagnosed from January 1990 to November 1999 in our hospital were analyzed retrospectively. Results In comparison with PNH cases reported in the 1980s, the newly diagnosed PNH cases showed the following features: (1) older age of disease onset (from 27 to 34 years); more female cases (from 18.5% to 38.5%); more cases without hemoglobinuria (from 24.2% to 38.5%). (2) No positive family hereditary history. (3) Bone marrow dysplasia, abnormal karyotype and negative sister chromatid differentiation were found in 19.2%, 12.2% and 8.9% of the PNH patients, respectively. 12.3% of the patients had bone marrow hypoplasia, and most of them had no hemoglobinuria. Ham's tests were negative in about 34.2% of the cases. CD55 and CD59 on peripheral blood cells were deficient in 100.0% of the cases, suggesting that CD55 and CD59 tests can improve the diagnosis of PNH. (4) Adrenocortical hormone was effective in 83.8% of the patients, 54.2% of whom relapsed within one year. Eight refractory and relapsed patients were treated with low dose chemotherapy (MP therapy: Melphalan 2-6?mg*d-1; Prednisone 0.5?mg*kg-1*d-1). Five (62.5%) of them showed positive responses. Bone marrow failure and other side effects were not serious in this group of patients. Conclusions PNH, an acquired blood disease seen more often among adult males, can be diagnosed more sensitively by hemocyte member CD55 and CD59 tests and treated more effectively with adrenocortical hormone or low dose chemotherapy.
