Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hematol. Nov 6, 2015; 4(4): 69-75
Published online Nov 6, 2015. doi: 10.5315/wjh.v4.i4.69
Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis: Study protocol of a prospective pilot study
Shinsaku Imashuku
Shinsaku Imashuku, Department of Laboratory Medicine, Uji-Tokushukai Medical Center, Uji 611-0042, Japan
Author contributions: Imashuku S wrote a paper and designed a pilot study protocol.
Conflict-of-interest statement: The author states no conflict of interest and has received no payment in preparation of this manuscript.
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:
Correspondence to: Shinsaku Imashuku, MD, PhD, Department of Laboratory Medicine, Uji-Tokushukai Medical Center, 145 Ishibashi, Makishima-cho, Uji 611-0042, Japan.
Telephone: +81-774-201111 Fax: +81-774-202336
Received: May 31, 2015
Peer-review started: June 4, 2015
First decision: August 14, 2015
Revised: September 6, 2015
Accepted: October 12, 2015
Article in press: October 13, 2015
Published online: November 6, 2015
Core Tip

Core tip: Diagnosis of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH) must fulfill both the evidence of EBV infection and the diagnostic criteria for HLH. EBV-HLH is heterogeneous. The majority of EBV-HLH occurs in apparently immunocompetent subjects, but some are associated with chronic active EBV infection status, X-linked lymphoproliferative disease or with familial HLH. Thus, treatment and outcome differ significantly depending on the underlying disease. To find out a most appropriate treatment, various laboratory tests are required to clarify the underlying diseases.