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Published in: Clinical Journal of Gastroenterology 6/2021

01-12-2021 | Computed Tomography | Case Report

TAFRO syndrome complicated by porto-sinusoidal vascular liver disease with portal hypertension: a case report

Authors: Manabu Hayashi, Jun Wada, Masashi Fujita, Tomoyuki Asano, Naoki Matsuoka, Yuya Fujita, Jumpei Temmoku, Haruki Matsumoto, Makiko Yashio-Furuya, Shuzo Sato, Hiroko Kobayashi, Hiroshi Watanabe, Kobashi Ryoichiro, Yuichi Waragai, Erina Suzuki, Yuichiro Kiko, Kazumichi Abe, Atsushi Takahashi, Tomoyuki Masuda, Yuko Hashimoto, Kiyoshi Migita, Hiromasa Ohira

Published in: Clinical Journal of Gastroenterology | Issue 6/2021

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Abstract

Porto-sinusoidal vascular liver disease (PSVD) is a disorder that can cause portal hypertension without liver cirrhosis. TAFRO syndrome is a systemic inflammatory disorder with a background of immunological abnormalities. We report a case of TAFRO syndrome complicated by PSVD with portal hypertension. A 39-year-old man developed refractory ascites and esophageal varices. Lymph node histology revealed multicentric Castleman disease-like features. Intravenous methylprednisolone and tocilizumab therapy improved ascites and renal dysfunction, but the patient developed severe infections. The diagnosis of TAFRO syndrome in patients complicated by PSVD with portal hypertension encourages the consideration of appropriate treatment for these patients.
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Metadata
Title
TAFRO syndrome complicated by porto-sinusoidal vascular liver disease with portal hypertension: a case report
Authors
Manabu Hayashi
Jun Wada
Masashi Fujita
Tomoyuki Asano
Naoki Matsuoka
Yuya Fujita
Jumpei Temmoku
Haruki Matsumoto
Makiko Yashio-Furuya
Shuzo Sato
Hiroko Kobayashi
Hiroshi Watanabe
Kobashi Ryoichiro
Yuichi Waragai
Erina Suzuki
Yuichiro Kiko
Kazumichi Abe
Atsushi Takahashi
Tomoyuki Masuda
Yuko Hashimoto
Kiyoshi Migita
Hiromasa Ohira
Publication date
01-12-2021
Publisher
Springer Singapore
Published in
Clinical Journal of Gastroenterology / Issue 6/2021
Print ISSN: 1865-7257
Electronic ISSN: 1865-7265
DOI
https://doi.org/10.1007/s12328-021-01515-2

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