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Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia

TAFRO 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 repo...

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Autores principales: Ono, Shiro, Yoshimoto, Kiyomi, Nishimura, Nobushiro, Yoneima, Ryo, Kawashima, Hiromasa, Kobayashi, Tadanao, Tai, Yoshiaki, Miyamoto, Makiko, Tsushima, Emiko, Yada, Noritaka, Nishio, Kenji
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8112990/
https://www.ncbi.nlm.nih.gov/pubmed/33191324
http://dx.doi.org/10.2169/internalmedicine.5850-20
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author Ono, Shiro
Yoshimoto, Kiyomi
Nishimura, Nobushiro
Yoneima, Ryo
Kawashima, Hiromasa
Kobayashi, Tadanao
Tai, Yoshiaki
Miyamoto, Makiko
Tsushima, Emiko
Yada, Noritaka
Nishio, Kenji
author_facet Ono, Shiro
Yoshimoto, Kiyomi
Nishimura, Nobushiro
Yoneima, Ryo
Kawashima, Hiromasa
Kobayashi, Tadanao
Tai, Yoshiaki
Miyamoto, Makiko
Tsushima, Emiko
Yada, Noritaka
Nishio, Kenji
author_sort Ono, Shiro
collection PubMed
description TAFRO 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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spelling pubmed-81129902021-05-18 Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia Ono, Shiro Yoshimoto, Kiyomi Nishimura, Nobushiro Yoneima, Ryo Kawashima, Hiromasa Kobayashi, Tadanao Tai, Yoshiaki Miyamoto, Makiko Tsushima, Emiko Yada, Noritaka Nishio, Kenji Intern Med Case Report TAFRO 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. The Japanese Society of Internal Medicine 2020-11-16 2021-04-15 /pmc/articles/PMC8112990/ /pubmed/33191324 http://dx.doi.org/10.2169/internalmedicine.5850-20 Text en Copyright © 2021 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Ono, Shiro
Yoshimoto, Kiyomi
Nishimura, Nobushiro
Yoneima, Ryo
Kawashima, Hiromasa
Kobayashi, Tadanao
Tai, Yoshiaki
Miyamoto, Makiko
Tsushima, Emiko
Yada, Noritaka
Nishio, Kenji
Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia
title Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia
title_full Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia
title_fullStr Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia
title_full_unstemmed Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia
title_short Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia
title_sort complete resolution of a case of tafro syndrome accompanied by mediastinal panniculitis, adrenal lesion, and liver damage with hyperbilirubinemia
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8112990/
https://www.ncbi.nlm.nih.gov/pubmed/33191324
http://dx.doi.org/10.2169/internalmedicine.5850-20
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