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Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging

Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It has an acute or subacute onset of u...

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Autores principales: Nara, Mizuho, Komatsuda, Atsushi, Itoh, Fumiko, Kaga, Hajime, Saitoh, Masaya, Togashi, Masaru, Kameoka, Yoshihiro, Wakui, Hideki, Takahashi, Naoto
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5491826/
https://www.ncbi.nlm.nih.gov/pubmed/28502946
http://dx.doi.org/10.2169/internalmedicine.56.7991
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author Nara, Mizuho
Komatsuda, Atsushi
Itoh, Fumiko
Kaga, Hajime
Saitoh, Masaya
Togashi, Masaru
Kameoka, Yoshihiro
Wakui, Hideki
Takahashi, Naoto
author_facet Nara, Mizuho
Komatsuda, Atsushi
Itoh, Fumiko
Kaga, Hajime
Saitoh, Masaya
Togashi, Masaru
Kameoka, Yoshihiro
Wakui, Hideki
Takahashi, Naoto
author_sort Nara, Mizuho
collection PubMed
description Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria. Our cases showed high serum procalcitonin levels, suggesting bacterial infection as an onset trigger. In addition, Case 1 is the first case complicated with adrenal hemorrhaging. Case 2 is the second case of tocilizumab-resistant TAFRO syndrome successfully treated with rituximab.
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spelling pubmed-54918262017-06-30 Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging Nara, Mizuho Komatsuda, Atsushi Itoh, Fumiko Kaga, Hajime Saitoh, Masaya Togashi, Masaru Kameoka, Yoshihiro Wakui, Hideki Takahashi, Naoto Intern Med Case Report Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis/Renal failure, and Organomegaly (TAFRO) syndrome is a recently described systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly. It has an acute or subacute onset of unknown etiology, although some pathological features resemble those of multicentric Castleman disease. We here report two cases of TAFRO syndrome. The symptoms and pathological findings in these cases met the 2015 diagnostic criteria. Our cases showed high serum procalcitonin levels, suggesting bacterial infection as an onset trigger. In addition, Case 1 is the first case complicated with adrenal hemorrhaging. Case 2 is the second case of tocilizumab-resistant TAFRO syndrome successfully treated with rituximab. The Japanese Society of Internal Medicine 2017-05-15 /pmc/articles/PMC5491826/ /pubmed/28502946 http://dx.doi.org/10.2169/internalmedicine.56.7991 Text en Copyright © 2017 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access article 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
Nara, Mizuho
Komatsuda, Atsushi
Itoh, Fumiko
Kaga, Hajime
Saitoh, Masaya
Togashi, Masaru
Kameoka, Yoshihiro
Wakui, Hideki
Takahashi, Naoto
Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging
title Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging
title_full Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging
title_fullStr Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging
title_full_unstemmed Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging
title_short Two Cases of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with High Serum Procalcitonin Levels, Including the First Case Complicated with Adrenal Hemorrhaging
title_sort two cases of thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly (tafro) syndrome with high serum procalcitonin levels, including the first case complicated with adrenal hemorrhaging
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5491826/
https://www.ncbi.nlm.nih.gov/pubmed/28502946
http://dx.doi.org/10.2169/internalmedicine.56.7991
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