We describe the case of a 23-month-old male infant with Epstein-Barr virus (EBV)-associated lymphoproliferative disorder, which mimicked the recurrence of EBV-associated hemophagocytic lymphohistiocytosis. Chemotherapy with dexamethasone, etoposide, and cyclosporine resolved fever, hepatosplenomegaly, and pancytopenia. However, on day 81 of illness, the patient developed similar symptoms. Plasma EBV-DNA levels markedly increased again, but no T-cell clonality was observed. B cells were identified to be infected with EBV. He was successfully treated with rituximab, dexamethasone and etoposide. When recurrence of EBV-associated hemophagocytic lymphohistiocytosis is suspected, performing tests to identify the infected cells will enable accurate understanding of the clinical condition, resulting in proper treatments.
*Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima City
†Department of Pediatrics, Shinshu University School of Medicine, Matsumoto
‡Department of Advanced Medicine for Infections, National Center for Child Health and Development
§Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
This work was supported in part by funds for Grant-in-Aid for Scientific Research (C) from the Ministry of Education, Culture, Sports, Science and Technology of Japan (Grant Number #16K10034 to TN).
The authors declare no conflict of interest.
Reprints: Takuro Nishikawa, MD, Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, 8-35-1 Sakuragaoka, Kagoshima City, 890-8520 Japan (e-mail: firstname.lastname@example.org).
Received August 8, 2017
Accepted November 8, 2017