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- Shiro Ono, Kiyomi Yoshimoto, Nobushiro Nishimura, Ryo Yoneima, Hiromasa Kawashima, Tadanao Kobayashi, Yoshiaki Tai, Makiko Miyamoto, Emiko Tsushima, Noritaka Yada, and Kenji Nishio.
- Department of General Medicine, Nara Medical University, Japan.
- Intern. Med. 2021 Apr 15; 60 (8): 1303-1309.
AbstractTAFRO syndrome is a systemic inflammatory, lymphoproliferative disorder, but the pathophysiology of the disease is unknown. It is typically characterized by thrombocytopenia, anasarca, a fever, reticulin fibrosis, renal dysfunction, and organomegaly. However, other manifestations have been also reported. We encountered a 43-year-old man with TAFRO syndrome who showed mediastinal panniculitis, liver damage, and adrenal lesions in addition to the core signs. He achieved complete remission with combination therapy of corticosteroids, tocilizumab, and cyclosporin, and remission was maintained even after drug discontinuation at 15 months. Atypical manifestations and complete remission of TAFRO syndrome were remarkable features of our case.
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