Korean J Med.  2015 Apr;88(4):464-468. 10.3904/kjm.2015.88.4.464.

Use of Splenectomy to Treat Evans Syndrome Following an ABO-Matched Liver Transplant

Affiliations
  • 1Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. khlee2@amc.seoul.kr
  • 2Division of Liver Transplantation and Hepatobiliary Surgery, Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Abstract

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.

Keyword

Autoimmune hemolytic anemia; Idiopathic thrombocytopenic purpura; Liver transplantation; Splenectomy

MeSH Terms

Adult
Anemia, Hemolytic, Autoimmune
Calcineurin
Graft vs Host Disease
Humans
Immunoglobulins
Liver Transplantation
Liver*
Purpura, Thrombocytopenic, Idiopathic
Splenectomy*
Rituximab
Calcineurin
Immunoglobulins
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