Budd-Chiari Syndrome Associated with Hypereosinophilic Syndrome; A Case Report

Ai Inoue; Kojiro Michitaka; Shuichiro Shigematsu; Ichiro Konishi; Masashi Hirooka; Yoichi Hiasa; Hidetaka Matsui; Bunzo Matsuura; Norio Horiike; Takaaki Hato; Hiroaki Miyaoka; Morikazu Onji

doi:10.2169/internalmedicine.46.6438

CASE REPORTS

Budd-Chiari Syndrome Associated with Hypereosinophilic Syndrome; A Case Report

Ai Inoue, Kojiro Michitaka, Shuichiro Shigematsu, Ichiro Konishi, Masashi Hirooka, Yoichi Hiasa, Hidetaka Matsui, Bunzo Matsuura, Norio Horiike, Takaaki Hato, Hiroaki Miyaoka, Morikazu Onji

Author information

Ai Inoue
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Kojiro Michitaka
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Shuichiro Shigematsu
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Internal Medicine, Saiseikai Matsuyama Hospital
Ichiro Konishi
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Masashi Hirooka
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Yoichi Hiasa
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Hidetaka Matsui
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Bunzo Matsuura
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Norio Horiike
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city
Takaaki Hato
Department of Bioregulatory Medicine, Ehime University Graduate School of Medicine, Toon-city
Hiroaki Miyaoka
Internal Medicine, Saiseikai Matsuyama Hospital
Morikazu Onji
Department of Gastroenterology and Metabology, Ehime University Graduate School of Medicine, Toon-city

Keywords: Budd-Chiari syndrome, hypereosinophilic syndrome, FIP1 like 1 (FIP1L1) gene, platelet-derived growth factor receptor α (PDGFRA) genes, obstructive thrombophlebitis

JOURNAL OPEN ACCESS

2007 Volume 46 Issue 14 Pages 1095-1100

DOI https://doi.org/10.2169/internalmedicine.46.6438

Details

Abstract

A 27-year-old man was admitted due to abdominal fullness. He had ascites and subcutaneous nodules on his head, with liver dysfunction and eosinophilia. Abdominal imaging revealed obstruction of the hepatic veins and stenosis of the inferior vena cava. Histological diagnosis of a subcutaneous nodule revealed obstructive thrombophlebitis with eosinophils. Tyrosine kinase created by fusion of the FIP1L1 and PDGFRA genes, which is characteristic of hypereosinophilic syndrome (HES), was detected. He was diagnosed with Budd-Chiari syndrome associated with HES. Liver function tests improved after interventional therapy followed by steroid therapy. It is important to diagnose the cause of Budd-Chiari syndrome.

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