非典型性尿毒溶血症候群病人之腎臟移植

Kidney Transplantation in Patients with Atypical Hemolytic Uremic Syndrome

蔡尚峰(Shang-Feng Tsai) ； 吳明儒(Ming-Ju Wu)

《腎臟與透析》 33卷2期 (2021/06) Pp. 105-109

https://doi.org/10.6340/KD.202106_33(2).0011

摘要

典型性尿毒溶血症候群（typical hemolytic uremic syndrome, HUS）是一種罕見的疾病，以微小血管內溶血性貧血（microangiopathic hemolytic anemia）、血小板減少症（＜ 150,000/μL）和急性腎臟損傷（acute kidney injury）為特徵的微血管阻塞性疾病。非典型HUS（atypical HUS, aHUS）為非ADAMTS13缺乏或是志賀氏毒素相關的HUS。aHUS造成的末期腎病而後移植，50%會復發且預後差。靜脈輸液、輸血、血漿交換、anti-CD20療法與同步肝移植都被報導過。Eeculizumab為針對補體C5的擬人化單株抗體，對aHUS需要腎臟移植時，正確診斷及治療，減少移植後接受透析的風險，並增加移植器官功能。

關鍵字

非典型性尿毒溶血症候群 ； 腎臟移植 ； 預後 ； eculizumab

並列摘要

Typical hemolytic uremic syndrome (HUS) is a rare disease, presented with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Atypical HUS (aHUS) is due to non-ADAMT13 deficiency or Shigatoxin-related HUS. The outcome of aHUS of post-renal transplantation is not good. Once occurred, in addition to hydration and transfusion, plasma exchange, anti-CD20 therapy, and liver transplantation were reported to be with benefits based on weak evidence. Recently, a new medication (eculizumab) is a monoclonal antibody that targets complement protein C5. The early detection of aHUS after renal transplantation and timely administration of eculizumab will obtain better graft outcomes.

並列關鍵字

atypical hemolytic uremic syndrome ； renal transplantation ； outcome ； eculizumab