Lucas Kenneth G, Ungar David, Comito Melanie, Groh Brandt
Division of Hematology/Oncology, Stem Cell Transplantation Program, Department of Pediatrics, Penn State Children's Hospital, 500 University Drive, C7830, MC H085, Hershey, Pennsylvania 17033, USA.
Pediatr Blood Cancer. 2008 May;50(5):1056-7. doi: 10.1002/pbc.21341.
IPEX syndrome (immune deficiency, polyendocrinopathy, enteropathy, X-linked) is a disorder or regulatory T cell (Treg) function which can result in early death due to infection or complications related to autoimmunity. Therapeutic options for these patients can include allogeneic stem cell transplantation (SCT) or the use of immunosuppressive regimens to control the manifestations of autoimmunity. We report a patient with IPEX syndrome who was managed with rapamycin and subsequently developed EBV induced lymphoma.
IPEX综合征（免疫缺陷、多内分泌腺病、肠病、X连锁）是一种调节性T细胞（Treg）功能障碍性疾病，可因感染或自身免疫相关并发症导致早期死亡。这些患者的治疗选择可包括异基因干细胞移植（SCT）或使用免疫抑制方案来控制自身免疫的表现。我们报告了一名接受雷帕霉素治疗的IPEX综合征患者，该患者随后发生了EB病毒诱导的淋巴瘤。
