Successful rituximab treatment of a patient with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis accompanied by X-linked lymphoproliferative syndrome type 1

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  • Hiroki Haruka
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Okano Tsubasa
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Yamashita Motoi
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Ashiarai Miho
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Miyamoto Satoshi
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Kobayashi Chika
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Aoki Yuki
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Takagi Masatoshi
    Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Imai Kohsuke
    Department of Community Pediatrics, Perinatal and Maternal Medicine, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Kanegane Hirokazu
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University
  • Morio Tomohiro
    Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University

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Other Title
  • Epstein-Barrウイルス関連血球貪食性リンパ組織球症にリツキシマブが奏功したX連鎖リンパ増殖症候群1型の一例

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<p>X-linked lymphoproliferative syndrome (XLP) is a rare primary immunodeficiency disease (PID) characterized by vulnerability to the Epstein-Barr virus (EBV). XLP is divided into XLP1 caused by SH2D1A mutation and XLP2 caused by XIAP mutation. Approximately 60% of patients with XLP1 present with EBV-associated hemophagocytic lymphohistiocytosis (HLH), which may be fatal despite treatment with etoposide and cyclosporine A. EBV mainly infects CD8+ T cells in sporadic EBV-HLH, whereas it mainly infects B cells in PIDs including XLP1. Treatment with an anti-CD20 monoclonal antibody (rituximab) may be effective in patients with EBV-HLH accompanied by XLP1. Here, we describe a 3-year-old boy with XLP1, who presented with severe EBV-HLH and was successfully treated with rituximab.</p>

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