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Korean J Med > Volume 88(4); 2015 > Article
The Korean Journal of Medicine 2015;88(4): 464-468.
ABO 일치 간 이식 후 발생한 Evans syndrome 증례보고
윤지현1, 안지환1, 조동희1, 김태은1, 송기원2, 이승규2, 이규형1
1울산대학교 의과대학 서울아산병원 내과
2울산대학교 의과대학 서울아산병원 간이식 및 간담도외과
Use of Splenectomy to Treat Evans Syndrome Following an ABO-Matched Liver Transplant
Ji Hyun Yun1, Jee Hwan Ahn1, Dong Hui Cho1, Taeeun Kim1, Gi-Won Song2, Sung-Gyu Lee2, Kyoo-Hyung Lee1
1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
2Department of Division of Liver Transplantation and Hepatobiliary Surgery, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Corresponding Author: Kyoo-Hyung Lee ,Tel: +82-2-3010-3213, Fax: +82-2-3010-6885, Email: khlee2@amc.seoul.kr
Received: May 7, 2014;   Revised: June 10, 2014;   Accepted: August 5, 2014.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Evans syndrome is a rare complication that develops in adults after liver transplantation. The possible etiologies include ABO mismatch, viral infection, post-transplantation lymphoproliferative disease, graft-versus-host disease, and the use of certain immunosuppressive drugs (e.g., calcineurin inhibitors). Here, we present a case of Evans syndrome that developed after an ABO-matched liver transplant. Glucocorticosteroid, intravenous immunoglobulin, and alternative immunosuppressant therapies all failed. Weekly rituximab (375 mg/m2) was then administered for 4 weeks. The cytopenia improved transiently after the second dose of rituximab, but soon worsened again. However, the cytopenia normalized after a splenectomy.
Keywords: Autoimmune hemolytic anemia; Idiopathic thrombocytopenic purpura; Liver transplantation; Splenectomy
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