-Short Communication-

Changes in Cytokine Profile during Initial Treatment of Pediatric Hemophagocytic Lymphohistiocytosis Associated with Epstein-Barr Virus

Takahiro Ueda, Toshikazu Itabashi, Shingo Yamanishi, Yujiro Tanabe, Makoto Migita and Yasuhiko Itoh

Department of Pediatrics, Nippon Medical School, Tokyo, Japan

Hemophagocytic lymphohistiocytosis (HLH) associated with Epstein-Barr virus (EBV) infection can be self-limiting, severe/aggressive, or fatal. We report a case of EBV-HLH with persistent fever, severe pancytopenia, hypertriglyceridemia, and hypofibrinogenemia in a 4-year-old boy. Levels of plasma cytokines and chemokines were measured with a Bio-Plex system at 1, 2, 3, 4, 5, and 8 days after hospital admission. Administration of steroid and high-dose intravenous immunoglobulin (1 g/kg) did not alleviate fever or reduce cytokine production; however, after administration of etoposide (an antineoplastic agent), fever decreased immediately, the patient's general condition improved, and levels of IL-6, IL-10, IL-8, MCP-1, IFN-γ, and TNF-α declined after etoposide administration. In particular, IFN-γ production sharply declined, from 1,104.1 pg/mL to 101.5 pg/mL, and IL-6 level decreased from 229.8 pg/mL to 11.0 pg/mL, on the day after initial etoposide administration. There was no later recurrence of symptoms during treatment with dexamethasone, etoposide, and cyclosporine A. This case suggests that early etoposide administration is critical for treatment success and indicates that etoposide promptly inhibits cytokine production.

J Nippon Med Sch 2020; 87: 166-170

Keywords
Epstein-Barr virus, hemophagocytic lymphohistiocytosis, immunochemotherapy, cytokine, etoposide

Correspondence to
Takahiro Ueda, MD, PhD, Department of Pediatrics, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
yuri878t@nms.ac.jp

Received, December 11, 2019
Accepted, February 6, 2020