Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 26, 2018; 6(14): 776-780
Published online Nov 26, 2018. doi: 10.12998/wjcc.v6.i14.776
Epstein–Barr virus-associated hemophagocytic syndrome in a patient with ulcerative colitis during treatment with azathioprine: A case report and review of literature
Kazuya Miyaguchi, Minoru Yamaoka, Yoshikazu Tsuzuki, Keigo Ashitani, Hideki Ohgo, Yoshitaka Miyagawa, Keisuke Ishizawa, Hidekazu Kayano, Hidetomo Nakamoto, Hiroyuki Imaeda
Kazuya Miyaguchi, Minoru Yamaoka, Yoshikazu Tsuzuki, Keigo Ashitani, Hideki Ohgo, Yoshitaka Miyagawa, Hidetomo Nakamoto, Hiroyuki Imaeda, Department of General Internal Medicine, Saitama Medical University, Iruma-gun 350-0495, Saitama, Japan
Yoshikazu Tsuzuki, Hideki Ohgo, Hiroyuki Imaeda, Department of Gastroenterology, Saitama Medical University, Iruma-gun 350-0495, Saitama, Japan
Keisuke Ishizawa, Hidekazu Kayano, Department of Pathology, Saitama Medical University, Iruma-gun 350-0495, Saitama, Japan
Author contributions: Miyaguchi K was involved in data collection, data analysis, and writing up the first draft of the paper; Tsuzuki Y contributed towards data analysis, and patient follow-up in the out-patient unit; Yamaoka M contributed to medical treatment; Imaeda H supervised, and finalized the manuscript; all authors approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the patient.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Yoshikazu Tsuzuki, FACG, MD, PhD, Associate Professor, Department of Gastroenterology, Saitama Medical University, 38 Morohongo, Moroyama-machi, Iruma-gun 350-0495, Saitama, Japan. ytsuzuki@saitama-med.ac.jp
Telephone: +81-49-2761667 Fax: +81-49-2761667
Received: June 22, 2018
Peer-review started: June 22, 2018
First decision: August 1, 2018
Revised: August 18, 2018
Accepted: October 8, 2018
Article in press: October 8, 2018
Published online: November 26, 2018
Abstract

A 19-year-old female was diagnosed with ulcerative colitis when she presented with persistent melena, and has been treated with 5-aminosalicylic acid for 4 years, with additional azathioprine for 2 years at our hospital. The patient experienced high-grade fevers, chills, and cough five d prior to presenting to the outpatient unit. At first, the patient was suspected to have developed neutropenic fever; however, she was diagnosed with Epstein-Barr virus-associated hemophagocytic syndrome (EB-VAHS) upon fulfilling the diagnostic criteria after bone marrow aspiration. When patients with inflammatory bowel disease treated with immunomodulators, such as thiopurine preparations, develop fever, EB-VAHS should be considered in the differential diagnosis.

Keywords: Inflammatory bowel disease, Azathioprine, Virus-associated hemophagocytic syndrome, Ulcerative colitis, Case report

Core tip: A 19-year-old female was diagnosed with ulcerative colitis, and has been receiving treatment with 5-aminosalicylic acid for 4 years, with additional azathioprine for 2 years. The patient experienced high-grade fever five d prior to presenting to the outpatient unit. She was diagnosed with Epstein-Barr virus-associated hemophagocytic syndrome (VAHS). VAHS is one of the rare and life-threatening pathophysiological conditions induced by thiopurine treatment. In such cases, early diagnosis is necessary, along with management of therapy, and related complications. When patients with inflammatory bowel disease, treated with immunomodulators such as thiopurine preparations, demonstrate high fever, VAHS should be considered in the differential diagnosis.