Epstein-Barr Virus-Induced Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is one of the complications of Epstein-Barr virus (EBV) infection. Although the patients who have developed HLH following EBV have normal immune system, there are a few patients with EBV-induced immune deficiency who develop HLH as well. Here, we describe the case of a 10-year-old girl with neurological complications caused by EBV-induced HLH. The patient received rituximab, leading to weakening inflammation associated with EBV infection and suppression of disease through quick treatment.

[1]  U. Stadt,et al.  Infection of T lymphocytes in Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis in children of non‐Asian origin , 2009, Pediatric blood & cancer.

[2]  M. Aricò,et al.  HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis , 2007, Pediatric blood & cancer.

[3]  H. Park,et al.  Successful Treatment of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis with HLH-94 Protocol , 2005, Journal of Korean medical science.

[4]  L. Silverman,et al.  Treatment of primary Epstein-Barr virus infection in patients with X-linked lymphoproliferative disease using B-cell-directed therapy. , 2004, Blood.

[5]  L. Mouthon,et al.  Pathogenesis of hemophagocytic syndrome (HPS). , 2004, Autoimmunity reviews.

[6]  T. Nakao,et al.  Treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center. , 2003, Medical and pediatric oncology.

[7]  M. Rowe,et al.  Epstein-Barr Virus Latent Membrane Protein-1 Mediates Upregulation of Tumor Necrosis Factor-α in EBV-Infected T Cells: Implications for the Pathogenesis of Hemophagocytic Syndrome , 2003, Journal of Biomedical Science.

[8]  H. Takada,et al.  Immunological aspects of Epstein-Barr virus infection. , 2002, Critical reviews in oncology/hematology.

[9]  S. Imashuku.  Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. , 2002, Critical reviews in oncology/hematology.

[10]  S. Hibi,et al.  Treatment Strategies for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis (EBV-HLH) , 2000, Leukemia & lymphoma.

[11]  Wan Ariffin Bin Abdullah,et al.  Med Pediatr Oncol , 1999 .

[12]  Jack R. Davis,et al.  Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene , 1998, Nature Genetics.

[13]  J. Chen,et al.  Upregulation of tumor necrosis factor-alpha gene by Epstein-Barr virus and activation of macrophages in Epstein-Barr virus-infected T cells in the pathogenesis of hemophagocytic syndrome. , 1997, The Journal of clinical investigation.

[14]  S. Hibi,et al.  Hemophagocytic lymphohistiocytosis in infancy and childhood , 1997 .

[15]  P. Lanzkowsky.  Manual of pediatric hematology and oncology , 1989 .