Thymoma-associated multiorgan autoimmunity with exclusive gastrointestinal tract involvement : case report and review of the literature
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Date
Authors
Slavik, Tomas
Potgieter, Fritz M.
Brittain, David
Journal Title
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Publisher
Springer
Abstract
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.
Description
Keywords
Thymoma-associated multiorgan autoimmunity (TAMA), Secondary autoimmune enteropathy, Graft-versus-host disease-like gastroenterocolitis, Paraneoplastic syndrome, Gastrointestinal tract (GIT), Skin, Liver
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Citation
Slavik, T., Potgieter, F.M. & Brittain, D. Thymoma-associated multiorgan autoimmunity with exclusive gastrointestinal tract involvement: case report and review of the literature. Virchows Archiv (2018) 473: 121-125. https://doi.org/10.1007/s00428-018-2304-6.