CAJ | 학술논문

回顾性总结北京协和医院2008年10月至2013年9月5年内共12例系统性红斑狼疮(SLE)相关血栓性血小板减少性紫癜(TTP)患者的临床资料.SLE相关TTP患者占同期住院SLE患者的0.45％(12/2 693).其中男女比例1∶5,中位年龄23岁.TTP诊断时疾病活动指数(SLEDAI)评分12 ～ 30分,多数为重度活动者(10/12).患者诊断前均有显著的血小板降低及明确肾损害.12例患者中10例出现急性肾衰竭;9例心肌受累;8例有中枢神经系统受累症状;发病时8例有4个及以上脏器损害;3例做了肾穿刺活检,Ⅳ型狼疮肾炎2例,Ⅴ型狼疮肾炎1例,病理均可见肾脏血栓性微血管病表现.外周血涂片中发现破碎红细胞是明确诊断的主要依据.11例接受了大剂量激素冲击治疗,6例最终好转(6/11).6例接受了血浆置换(40 ml· kg-1·d-1),4例好转.未接受血浆置换的6例,3例好转.12例患者最终死亡2例,恶化放弃治疗3例,7例好转.
회고성총결북경협화의원2008년10월지2013년9월5년내공12례계통성홍반랑창(SLE)상관혈전성혈소판감소성자전(TTP)환자적림상자료.SLE상관TTP환자점동기주원SLE환자적0.45％(12/2 693).기중남녀비례1∶5,중위년령23세.TTP진단시질병활동지수(SLEDAI)평분12 ～ 30분,다수위중도활동자(10/12).환자진단전균유현저적혈소판강저급명학신손해.12례환자중10례출현급성신쇠갈;9례심기수루;8례유중추신경계통수루증상;발병시8례유4개급이상장기손해;3례주료신천자활검,Ⅳ형랑창신염2례,Ⅴ형랑창신염1례,병리균가견신장혈전성미혈관병표현.외주혈도편중발현파쇄홍세포시명학진단적주요의거.11례접수료대제량격소충격치료,6례최종호전(6/11).6례접수료혈장치환(40 ml· kg-1·d-1),4례호전.미접수혈장치환적6례,3례호전.12례환자최종사망2례,악화방기치료3례,7례호전.
We retrospectively reviewed the clinical records of 12 systemic lupus erythematosus (SLE) patients with thrombotic thrombocytopenic purpura (TTP) admitted into Peking Union Medical College Hospital (PUMCH) during October 2008 to September 2013.TTP occurred in 0.45％ (12/2 693) of patients with SLE.The median age (2 males,10 females) was 23 years.Their SLEDAI scores ranged from 12 to 30.All patients had significant thrombocytopenia and renal abnormalities.Ten of them bad acute renal failure.Cardiac involvement and neurological deficits were observed in 9 and 8 of them respectively.Eight of them had injuries of more than 4 organs.Renal biopsy of 3 patients revealed lupus nephritis (type Ⅳ,n =2 ; type Ⅴ,n =1) and thrombotic microangiopathy.Eleven TTP patients received steroid pulse therapy (1 g/d,3 days) and six of them survived.Six patients were rescued by therapeutic plasma exchange and 4 of them recovered.Among another 6 patients not on plasma exchange therapy,only 3 recovered.Finally 2 died,3 gave up with exacerbated symptoms after therapy and 7 survived.