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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...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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The Japanese Society of Internal Medicine
2020
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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. |
format | Online Article Text |
id | pubmed-8112990 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | The Japanese Society of Internal Medicine |
record_format | MEDLINE/PubMed |
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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