title: Clinical features and treatment strategies of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
abstract: Epstein-Barr virus (EBV) is the major triggering factor producing hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH). In this review, diagnostic problems, clinical and histopathological features, and treatment strategies of EBV-HLH have been described. In patients with EBV-HLH, the EBV-infected T cells or natural killer (NK) cells are mostly mono- or oligo-clonally proliferating, where hypercytokinemia plays a major role and causes hemophagocytosis, cellular damage and dysfunction of various organs. Although the majority of EBV-HLH cases develop in apparently immunocompetent children and adolescents, it also occurs in association with infectious mononucleosis, chronic active EBV infection, familial HLH, X-linked lymphoproliferative disease, lymphoproliferative disease like peripheral T-cell lymphoma and NK cell leukemia. In terms of treatment, special therapeutic measures are required to control the cytokine storm generated by EBV and to suppress proliferating EBV-genome-containing cells, because the clinical courses are often fulminant and result in a poor outcome.
authors.affiliation: Kyoto City Institute of Health and Environmental Sciences, 1-2 Higashi-Takada-cho, Mibu, Nakagyo-ku, Kyoto 604-8854, Japan. shinim95@mbox.kyoto-inet.or.jp
authors.forename: Shinsaku
authors.initials: S
authors.lastname: Imashuku
mesh_terms: D000998:Antiviral Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D016207:Cytokines / Q000378:metabolism; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D018380:Hematopoietic Stem Cell Transplantation; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007167:Immunotherapy / Q000379:methods; D007694:Killer Cells, Natural / Q000821:virology; D013601:T-Lymphocytes / Q000821:virology
chemical_list: D000998:Antiviral Agents; D016207:Cytokines