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

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.

• 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
• Journal: Clinical journal of gastroenterology (Clin J Gastroenterol) Vol. 14 Issue 6 Pg. 1711-1717 (Dec 2021) ISSN: 1865-7265 [Electronic] Japan
• PMID: 34487333 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2021. Japanese Society of Gastroenterology.

Topics

• Adult
• Castleman Disease
• Humans
• Hypertension, Portal (complications)
• Liver Cirrhosis (complications)
• Male
• Vascular Diseases