Orfanoudaki Eleni, Iliadis Alexandros, Goulis Ioannis, Xylouri Eirini, Voumvouraki Argyro, Samonakis Dimitrios N
From the Department of Gastroenterology and Hepatology, Liver Transplantation and Liver Cancer Clinic, University Hospital of Heraklion, Crete, Greece.
Exp Clin Transplant. 2021 Jun;19(6):620-623. doi: 10.6002/ect.2021.0015.
Immunoproliferative small intestinal disease is an extranodal marginal zone B-cell lymphoma that arises from mucosa-associated lymphoid tissue and is associated with defective α heavy chain protein secretion. We present a case of an 18-year-old male patient admitted with diarrhea and weight loss who had previously received a liver transplant at the age of 19 months to treat biliary atresia. He underwent a thorough investigation and was diagnosed with immunoproliferative small intestinal disease lymphoma. The patient was switched from tacrolimus to everolimus and commenced on doxycycline treatment for 6 months and achieved long-term remission. Currently, 7 years after diagnosis, he is asymptomatic without evidence of histological relapse. This is the first case of immunoproliferative small intestinal disease described in a liver transplant recipient.
免疫增殖性小肠疾病是一种结外边缘区B细胞淋巴瘤，起源于黏膜相关淋巴组织，与α重链蛋白分泌缺陷有关。我们报告一例18岁男性患者，因腹泻和体重减轻入院，该患者曾在19个月大时接受肝移植以治疗胆道闭锁。他接受了全面检查，被诊断为免疫增殖性小肠疾病淋巴瘤。患者从他克莫司换成依维莫司，并开始接受强力霉素治疗6个月，实现了长期缓解。目前，诊断后7年，他无症状，无组织学复发证据。这是肝移植受者中描述的首例免疫增殖性小肠疾病病例。
