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成人血栓性血小板减少性紫癜/溶血尿毒综合征患者的肾移植

Renal transplantation in adults with thrombotic thrombocytopenic purpura/haemolytic-uraemic syndrome.

作者信息

Conlon P J, Brennan D C, Pfaf W W, Finn W F, Gehr T, Bollinger R R, Smith S R

机构信息

Department of Medicine, Duke University Medical Center, Durham, NC 27710, USA.

出版信息

Nephrol Dial Transplant. 1996 Sep;11(9):1810-4.

PMID:8918627
Abstract

BACKGROUND

Thrombotic thrombocytopenic purpura/haemolytic-uraemic syndrome (TTP/HUS) is a rare cause of renal failure in adults. There is little data concerning the outcome of adult patients who receive a renal transplant for TTP/HUS:

METHODS

We have carried out a survey of 22 transplant centres in the USA to determine the outcome of patients who developed ESRD from TTP/HUS and latter received a renal transplant.

RESULTS

Twelve of the 22 centres responded to our inquiry. Seven centres had not transplanted any patients with TTP/HUS, and five centres had transplanted a total of 24 grafts in 17 patients with TTP/HUS: Thirty-three per cent of patients demonstrated definite clinical and pathological evidence of recurrence of TTP/HUS: An additional 16% of patients demonstrated pathological evidence of possible recurrence of TTP/HUS in the absence of clinical manifestations. The overall 1-year graft survival rate was 42% and the 2-year graft survival rate was 35%. In our experience recurrence TTP/HUS was associated with universal graft failure. Although cyclosporin A does occasionally cause a thrombotic angiopathy in patients with no history of TTP/HUS, we found no evidence that it should be avoided in patients with a previous history of ESRD from TTP/HUS who subsequently receive a renal transplant.

CONCLUSIONS

TTP/HUS frequently recurres in adults who receive a renal transplant, with a 2-year graft survival rate of 35%.

摘要

背景

血栓性血小板减少性紫癜/溶血尿毒综合征(TTP/HUS)是成人肾衰竭的罕见病因。关于因TTP/HUS接受肾移植的成年患者的预后数据很少。

方法

我们对美国22个移植中心进行了一项调查,以确定因TTP/HUS发展为终末期肾病(ESRD)并随后接受肾移植的患者的预后。

结果

22个中心中有12个回应了我们的询问。7个中心没有移植过任何TTP/HUS患者,5个中心共为17例TTP/HUS患者移植了24个移植物。33%的患者有TTP/HUS复发的确切临床和病理证据,另有16%的患者在无临床表现的情况下有TTP/HUS可能复发的病理证据。总体1年移植物存活率为42%,2年移植物存活率为35%。根据我们的经验,TTP/HUS复发与移植物普遍失败相关。虽然环孢素A偶尔会在无TTP/HUS病史的患者中引起血栓性血管病,但我们没有发现证据表明,对于既往因TTP/HUS导致ESRD且随后接受肾移植的患者应避免使用环孢素A。

结论

接受肾移植的成人中TTP/HUS经常复发,2年移植物存活率为35%。

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