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EVANS SYNDROME AFTER SUCCESSFUL IMMUNOSUPPRESSANT-FREE LIVING-DONOR LIVER TRANSPLANT

AMR BADAWY, TOSHİMİ KAİDO, YOSHİZAWA ATSUSHİ, SHİNTARO YAGİ, KOİCHİRO HATA, NAOKO KAMO, KEN FUKUMİTSU, HİDEAKİ OKAJİMA, SHİNJİ UEMOTO

Experimental and Clinical Transplantation - 2020;18(2):258-260

Hepato-Biliary-Pancreatic Surgery and Transplantation Department, Kyoto University, Kyoto, Japan

 

Evans syndrome is an uncommon disease charac - terized by a combination of autoimmune hemolytic anemia and autoimmune thrombocytopenia con - comitantly or sequentially with a positive direct Coombs test in the absence of any underlying known cause. Here, we present a case of an adult patient who underwent living-donor liver transplant that was preceded by bone marrow transplant 20 years earlier from the same HLA identical donor and who received a single-agent immunosuppressive therapy for only 2 months as prophylaxis against graft-versus-host disease. Two months after transplant, he developed Evans syndrome with severe anemia and throm - bocytopenia. After administration of steroids and intravenous immunoglobulin, the patient’s anemia and thrombocytopenia improved dramatically. Through the 7 years of follow-up, the patient has not developed graft-versus-host disease or acute or chronic rejection. This case demonstrates a rare complication post - transplant and the possibility of functional tolerance of liver grafts after a combined liver and bone marrow transplant from the same donor.