Tohoku J. Exp. Med., 2008, 214(1)

Successful Treatment of Neonatal Herpes Simplex-Type 1 Infection Complicated by Hemophagocytic Lymphohistiocytosis and Acute Liver Failure

KEITARO YAMADA,^1,2 YUTAKA YAMAMOTO,^1,2 ATSUSHI UCHIYAMA,¹ REIKO ITO,³ YUSUKE AOKI,^2,4 YASUSHI UCHIDA,^2,4 HIROYUKI NAGASAWA,¹ HIROSHI KIMURA,⁵ TAKASHI ICHIYAMA,⁶ TOSHIYUKI FUKAO² and YOSHINORI KOHNO¹

¹Department of Neonatology, Gifu Prefectural General Medical Center, Gifu, Japan
²Department of Pediatrics, Graduate School of Medicine, Gifu University, Gifu, Japan
³Department of Pediatrics, Gifu Prefectural General Medical Center, Gifu, Japan
⁴Department of Pediatrics, Nagara Medical Center, Gifu, Japan
⁵Department of Microbiology, Graduate School of Medicine, Nagoya University, Nagoya, Japan
⁶Department of Pediatrics, Graduate School of Medicine, Yamaguchi University, Yamaguchi, Japan

Neonatal disseminated herpes simplex virus (HSV) infection with acute liver failure (ALF) and neonatal hemophagocytic lymphohistiocytosis (HLH) are severe diseases. We recently experienced a male infant with HLH and ALF induced by HSV type 1 (HSV-1). The infant, born at 39 weeks of gestation by normal delivery, developed a fever on day 4. On day 9, laboratory investigations showed progressive liver dysfunction and coagulopathy, and the serum ferritin was excessively elevated. Furthermore, the blood levels of interleukin (IL)-6, IL-10, and interferon-gamma were also elevated. HSV-1 DNA was detected in the serum and cerebrospinal fluid by the real-time PCR method. A diagnosis of HLH was established based upon the following criteria: fever, splenomegaly, cytopenia (two cell lines), serum ferritin (> 500 μg/l) and hypofibrinogenemia (< 150 mg/dl). High-dose acyclovir therapy, steroid pulse therapy using methylprednisolone, high-dose gamma globulin therapy and a blood transfusion were given. The patient recovered without neurological deficit. Neonatal disseminated HSV infections may be complicated by the development of HLH and hypercyokinemia. If HLH is suspected, not only high-dose acyclovir therapy but also anti-cytokine therapy should be considered.

keywords —— newborn; herpes simplex virus type 1 infection; hemophagocytic lymphohistiocytosis; hypercytokinemia; acute liver failure.

© 2008 Tohoku University Medical Press

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Tohoku J. Exp. Med., 2008, 214, 1-5

Correspondence: Keitaro Yamada, Department of Pediatrics, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu 501-1194, Japan.

e-mail: keitaro_ymd1976@yahoo.co.jp