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Case Report
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a ten-year-old girl accompanied by posterior reversible encephalopathy syndrome and disseminated herpes simplex virus infection

Ryuta Itakura^＊, Fumihiko Namba, Ayumu Arakawa,
Yoshitaka Asano, Koichi Moriwaki, Masanori Tamura
Department of Pediatrics, Saitama Medical Center, Saitama Medical University

　Posterior reversible encephalopathy syndrome (PRES) has become a common disease entity. Clinically, the patient usually presents with acute onset of neurological problems including headache, convulsions and visual impairment or blindness. On the other hand, disseminated herpes simplex virus (HSV)-1 infection is usually observed in immunocompromised individuals. We present a case of cyclosporin A (CyA)-induced PRES and disseminated HSV-1 infection during chemotherapy treatment with etoposide, prednisolone and CyA for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH). The combination therapy for EBV-HLH was initially effective. However, the patient developed prominent side effects, drug-induced encephalopathy and the risk of infection under an immunosuppressive state. She showed an excellent outcome after discontinuation of CyA and treatment with acyclovir and methylprednisolone. In conclusion, this case report emphasizes the difficulty in choosing an appropriate treatment for EBV-HLH when some side effects develop and the importance of making a timely diagnosis for preventing progression to permanent damage from HLH-associated encephalopathy or PRES.

J Saitama Medical University 2015; 41(2): 139-143
(Received August 7, 2014 / Accepted November 28, 2014)

Keywords: Epstein-Barr virus, hemophagocytic lymphohistiocytosis, cyclophosphamide, posterior reversible encephalopathy syndrome, herpes simplex virus-1