Article in Japanese

A case of EBV-associated hemophagocytic lymphohistiocytosis with proliferation of virus-infected B cells on relapse

Hitomi KITANO¹⁾, Yosuke FUJII¹⁾, Nobuyuki NOSAKA¹⁾, Masato YASHIRO¹⁾, Akira SHIMADA¹⁾, Hirokazu TSUKAHARA¹⁾

This study presented a 2-year-old girl with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH); i.e. EBV infecting her CD8 positive T-cells on disease onset, and being detected preferentially in her B-cells on relapse. She was admitted to this hospital with fever and pancytopenia, and received a diagnosis of EBV-HLH. The EBV genome copy number was monitored in the infected cells by real-time PCR in order to assess disease activity and response to therapy. The EBV genome was detected initially in the T cells. Treatment was started based on the hemophagocytic lymphohistiocytosis (HLH) -2004 protocol during which EBV genome copies became undetectable temporarily, but 1.14×10⁵ copies/ml of the EBV genome were detected at the end of treatment. The EBV genome was detected preferentially in B cells at re-examination. EBV-HLH relapse usually requires hematopoietic stem cell transplantation; however, rituximab is thought to be effective for EBV infection in B cells. After rituximab treatment, EBV genome copies became undetectable persistently. Monitoring the EBV genome copy number and infected cells may be useful in treatment decisions for EBV-HLH.

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1) Department of Pediatrics, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences

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Received	February 6, 2017
Accepted	May 8, 2017

29 (2)：165─170,2017