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The first successful eculizumab rescue therapy of a kidney transplant recipient with atypical hemolytic uremic syndrome in South Korea: a case report

Authors
 Eun-Ki Min  ;  Hyun Jeong Kim  ;  Sinyoung Kim  ;  Minsun Jung  ;  Jin Seok Kim  ;  Seung Hyeok Han  ;  Kyu Ha Huh 
Citation
 Korean Journal of Transplantation, Vol.37(1) : 57-62, 2023-03 
Journal Title
Korean Journal of Transplantation
ISSN
 2671-8790 
Issue Date
2023-03
Keywords
Atypical hemolytic uremic syndrome ; Case report ; Eculizumab ; Kidney transplantation ; Thrombotic microangiopathy
Abstract
Atypical hemolytic uremic syndrome (aHUS) is a form of thrombotic microangiopathy (TMA) that can result in end-stage renal disease. Patients with aHUS often have predisposing dysfunction in the complement pathway, and continuous activation of complement proteins can be triggered after transplantation. Here, we report the first successful case of aHUS treatment in a kidney transplant recipient with early use of a C5 inhibitor, eculizumab, in South Korea. The patient was a 32-year-old man, and the donor was his 60-year-old mother. The graft showed immediate good function. On postoperative day (POD) 3, the clinical diagnosis of TMA was made. Persistent renal dysfunction despite 10 plasma exchange (PE) sessions prompted eculizumab treatment on POD 18 under suspicion of aHUS. Next-generation sequencing reported gene mutations classified as variants of unknown significance in coagulation-associated genes. The patient was discharged after three doses of eculizumab with serum creatinine of 1.82 mg/dL. In total, 16 doses of eculizumab were administered. At the last follow-up, 21 months after eculizumab discontinuation, the graft was well functioning. De novo TMA after kidney transplantation can be caused by sustained activation of the complement pathway, and early eculizumab treatment appears important in the successful treatment of aHUS refractory to PE.
Files in This Item:
T202306328.pdf Download
DOI
10.4285/kjt.22.0050
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Laboratory Medicine (진단검사의학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Surgery (외과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Sin Young(김신영) ORCID logo https://orcid.org/0000-0002-2609-8945
Kim, Jin Seok(김진석) ORCID logo https://orcid.org/0000-0001-8986-8436
Kim, Hyun Jeong(김현정)
Min, Eun-Ki(민은기)
Jung, Minsun(정민선) ORCID logo https://orcid.org/0000-0002-8701-4282
Han, Seung Hyeok(한승혁) ORCID logo https://orcid.org/0000-0001-7923-5635
Huh, Kyu Ha(허규하) ORCID logo https://orcid.org/0000-0003-1364-6989
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/196785
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