Gonçalves Cristina, Ferreira Sandra, Nobre Susana, Gonçalves Isabel
Unidade de Transplantação Hepática - PA, Centro Hospitalar e Universitario de Coimbra EPE, Coimbra, Portugal.
BMJ Case Rep. 2017 Dec 13;2017:bcr-2017-221434. doi: 10.1136/bcr-2017-221434.
Prognosis of hepatitis-associated aplastic anaemia (HAAA) was improved with haematopoietic stem cell transplantation (HSCT) and immunosuppression, but the long-term outcome remains undefined. Case 1: a girl aged 3 years with acute liver failure (ALF) submitted to orthotopic liver transplantation (OLT) subsequently developed aplastic anaemia and HSCT from a compatible sibling was performed. Post-HSCT, the patient developed post-transplant lymphoproliferative disorder and rituximab was administered with good response. Fifteen years later, both grafts show good outcome. Case 2: a girl aged 10 years submitted to OLT due to ALF, developed pancytopenia 2 months later. Due to the absence of a human leucocyte antigen compatible donor, she was treated with ciclosporin and antithymocyte globulin with very good long-term outcome. These clinical cases suggest that, for patients with HAAA that underwent OLT, aggressive therapy with HSCT or immunosuppression may provide a benign long-term outcome.
造血干细胞移植（HSCT）和免疫抑制改善了肝炎相关性再生障碍性贫血（HAAA）的预后，但长期结局仍不明确。病例1：一名3岁女童因急性肝衰竭（ALF）接受原位肝移植（OLT），随后发生再生障碍性贫血，并接受了来自相容同胞的HSCT。HSCT后，患者发生移植后淋巴细胞增殖性疾病，给予利妥昔单抗治疗，反应良好。15年后，两个移植物均显示良好结局。病例2：一名10岁女童因ALF接受OLT，2个月后出现全血细胞减少。由于缺乏人类白细胞抗原相容供体，她接受了环孢素和抗胸腺细胞球蛋白治疗，长期结局非常好。这些临床病例表明，对于接受OLT的HAAA患者，积极进行HSCT或免疫抑制治疗可能带来良好的长期结局。
