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肾移植后血栓性微血管病:一种诊断不足且可能可逆的病症。

Thrombotic Microangiopathy After Kidney Transplantation: An Underdiagnosed and Potentially Reversible Entity.

作者信息

Ávila Ana, Gavela Eva, Sancho Asunción

机构信息

Nephrology Department, University Hospital Dr. Peset, Valencia, Spain.

出版信息

Front Med (Lausanne). 2021 Apr 8;8:642864. doi: 10.3389/fmed.2021.642864. eCollection 2021.

Abstract

Thrombotic microangiopathy is a rare but serious complication that affects kidney transplant recipients. It appears in 0.8-14% of transplanted patients and negatively affects graft and patient survival. It can appear in a systemic form, with hemolytic microangiopathic anemia, thrombocytopenia, and renal failure, or in a localized form, with progressive renal failure, proteinuria, or arterial hypertension. Post-transplant thrombotic microangiopathy is classified as recurrent atypical hemolytic uremic syndrome or thrombotic microangiopathy. thrombotic microangiopathy accounts for the majority of cases. Distinguishing between the 2 conditions can be difficult, given there is an overlap between them. Complement overactivation is the cornerstone of all post-transplant thrombotic microangiopathies, and has been demonstrated in the context of organ procurement, ischemia-reperfusion phenomena, immunosuppressive drugs, antibody-mediated rejection, viral infections, and post-transplant relapse of antiphospholipid antibody syndrome. Although treatment of the causative agents is usually the first line of treatment, this approach might not be sufficient. Plasma exchange typically resolves hematologic abnormalities but does not improve renal function. Complement blockade with eculizumab has been shown to be an effective therapy in post-transplant thrombotic microangiopathy, but it is necessary to define which patients can benefit from this therapy and when and how eculizumab should be used.

摘要

血栓性微血管病是一种罕见但严重的并发症,影响肾移植受者。它出现在0.8%-14%的移植患者中,对移植物和患者的存活产生负面影响。它可以以全身形式出现,伴有微血管病性溶血性贫血、血小板减少和肾衰竭,或以局部形式出现,伴有进行性肾衰竭、蛋白尿或动脉高血压。移植后血栓性微血管病被分类为复发性非典型溶血性尿毒症综合征或血栓性微血管病。血栓性微血管病占大多数病例。鉴于两者之间存在重叠,区分这两种情况可能很困难。补体过度激活是所有移植后血栓性微血管病的基石,并且已在器官获取、缺血再灌注现象、免疫抑制药物、抗体介导的排斥反应、病毒感染以及抗磷脂抗体综合征的移植后复发的背景下得到证实。虽然治疗致病因素通常是一线治疗方法,但这种方法可能并不足够。血浆置换通常可解决血液学异常,但不能改善肾功能。已证明用依库珠单抗进行补体阻断是移植后血栓性微血管病的有效治疗方法,但有必要确定哪些患者可以从这种治疗中获益,以及何时以及如何使用依库珠单抗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/46a0/8063690/9a9bf8a569db/fmed-08-642864-g0001.jpg

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