Thymoma-associated multiorgan autoimmunity with exclusive gastrointestinal tract involvement: case report and review of the literature
Thymoma-associated multiorgan autoimmunity with exclusive gastrointestinal tract involvement: case report and review of the literature
复制标题
胸腺瘤相关多器官自身免疫仅累及胃肠道：病例报告和文献综述
作者：
T. Slavik;Fritz M Potgieter;D. Brittain
胸腺瘤相关多器官自身免疫（TAMA）是最近在胸腺瘤患者中报道的一种罕见的副肿瘤综合征。这种疾病的特点是移植物抗宿主病样病理影响皮肤、胃肠道（GIT）和肝脏，通常伴有不良预后。我们记录了一例TAMA，包括胃，小肠和大肠，在完全切除B2型胸腺瘤5年后出现的66岁男性患者。本文简要回顾了这种罕见综合征，迄今为止报道的21例TAMA病例中描述的GIT病理，以及这种获得性自身免疫性疾病仅涉及GIT的患者的独特特征。
Thymoma-associated multiorgan autoimmunity (TAMA) is a recently delineated and rare paraneoplastic syndrome reported in patients with thymoma. The disorder is characterized by graft-versus-host disease-like pathology affecting the skin, gastrointestinal tract (GIT), and liver, and is usually associated with a poor outcome. We document a case of TAMA with exclusive GIT involvement which included the stomach, small and large bowel, presenting in a 66-year-old male patient 5 years after complete resection of a type B2 thymoma. A brief review is provided of this scarce syndrome, the GIT pathology described in the 21 TAMA cases reported to date, and the unique characteristics of patients with exclusive GIT involvement by this acquired autoimmune disorder.