Acquazzino Melissa A, Fischer Ryan T, Langnas Alan, Coulter Don W
Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE 68198-2168, USA.
Pediatr Transplant. 2013 Aug;17(5):466-71. doi: 10.1111/petr.12101. Epub 2013 Jun 3.
AIHA is a rare and serious complication of solid organ transplantation. Herein, we report four cases of warm or mixed AIHA in pediatric patients following combined liver, small bowel and pancreas transplant. The hemolysis was refractory to multiple treatment modalities including steroids, rituximab, IVIG, plasmapheresis, cytoxan, discontinuation of prophylactic penicillin, and a change in immunosuppression from tacrolimus to cyclosporine. All patients had resolution or marked improvement of hemolysis after discontinuation of maintenance of CNI and initiation of sirolimus immunosuppression. One patient developed nephrotic syndrome but responded to a change in immunosuppression to everolimus. Three of the four patients continue on immunosuppression with sirolimus or everolimus without further hemolysis, evidence of rejection or medication side effects. Based on our experience and review of similar cases in the literature, we have proposed a treatment algorithm for AIHA in the pediatric intestinal transplant patient population that recommends an early change in immunosuppressive regimen from CNIs to sirolimus therapy.
自身免疫性溶血性贫血（AIHA）是实体器官移植中一种罕见且严重的并发症。在此，我们报告4例小儿患者在接受肝脏、小肠和胰腺联合移植后发生温抗体型或混合型AIHA的病例。溶血对多种治疗方式均无效，这些治疗方式包括使用类固醇、利妥昔单抗、静脉注射免疫球蛋白（IVIG）、血浆置换、环磷酰胺、停用预防性青霉素以及将免疫抑制剂从他克莫司换成环孢素。在停用钙调神经磷酸酶抑制剂（CNI）维持治疗并开始使用西罗莫司进行免疫抑制后，所有患者的溶血均得到缓解或显著改善。1例患者出现肾病综合征，但在将免疫抑制剂换成依维莫司后有反应。4例患者中有3例继续使用西罗莫司或依维莫司进行免疫抑制，未再发生溶血、排斥反应迹象或药物副作用。基于我们的经验以及对文献中类似病例的回顾，我们提出了一种针对小儿肠道移植患者群体中AIHA的治疗方案，建议早期将免疫抑制方案从CNI换成西罗莫司治疗。
