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Evans Syndrome After Successful Immunosuppressant-Free Living-Donor Liver Transplant

机译:evans综合征成功免疫抑制剂的无生活肝脏移植

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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.
机译:evans综合征是一种罕见的疾病charac? - 通过自身免疫性溶血性贫血和自身免疫血小板减少症的组合,在没有任何潜在的已知原因的情况下用阳性直接组织试验依次或顺序地依次。在这里,我们提出了一种成年患者,该患者接受了20年前的骨髓移植前面的患者肝脏移植,同样来自同一HLA相同的供体,他只接受了单孕免疫抑制治疗,只有2个月作为抗移植物的预防 - 宿主病。移植后两个月,他开发了evans综合征,具有严重的贫血和血统? - 细胞多脑。在给药类固醇和静脉内免疫球蛋白后,患者的贫血和血小板减少症急剧提高。通过7年的随访,患者尚未开发接枝腹膜疾病或急性或慢性排斥反应。本例证明了罕见的并发症术后α-植物和肝移植后的功能耐受性,在同一供体中移植后肝脏移植物。

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