title: T/NK cell type chronic active Epstein-Barr virus disease in adults: an underlying condition for Epstein-Barr virus-associated T/NK-cell lymphoma.
abstract: A chronic infectious mononucleosis-like illness caused by Epstein-Barr virus (EBV) is called 'chronic active EBV disease', which is defined as an EBV-associated lymphoproliferative disease. This lymphoproliferative disease is rare and predominantly occurs in Japanese children. Between 1998 and 2010, seven adult-onset cases (aged 20-45 years, median 39 years) were identified, which initially presented with inflammatory diseases, including hepatitis, interstitial pneumonitis, uveitis, nephritis and hypersensitivity to mosquito bites. They showed an EBV viral load in the peripheral blood and evidence of EBV infection of T or natural killer (NK) cells. Five cases (71.4%) developed EBV-positive T/NK-cell lymphoma/leukaemia at a median of 5 years (range 1-7 years) after the diagnosis. Although l-asparaginase-containing chemotherapy was effective for the lymphomas, only allogeneic haematopoietic cell transplantation eradicated EBV-infected cells. This observation indicates that persistent EBV infection of T or NK cells defines a distinct disease entity, which provides an underlying condition for EBV-positive T/NK-cell lymphoma/leukaemia.
authors.affiliation: Department of Hematology, Juntendo University School of Medicine, Bunkyo-ku, Tokyo, Japan. yisobe@juntendo.ac.jp
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Isobe
authors.forename: Nanae
authors.initials: N
authors.lastname: Aritaka
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Setoguchi
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Ito
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kimura
authors.forename: Yasuharu
authors.initials: Y
authors.lastname: Hamano
authors.forename: Koichi
authors.initials: K
authors.lastname: Sugimoto
authors.forename: Norio
authors.initials: N
authors.lastname: Komatsu
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000008:administration & dosage; D001706:Biopsy; D015139:Blotting, Southern; D002908:Chronic Disease; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000628:therapy / Q000821:virology*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007150:Immunohistochemistry; D007694:Killer Cells, Natural / Q000276:immunology / Q000821:virology*; D054391:Lymphoma, Extranodal NK-T-Cell / Q000175:diagnosis / Q000276:immunology / Q000628:therapy / Q000821:virology*; D008297:Male; D008875:Middle Aged; D013601:T-Lymphocytes / Q000276:immunology / Q000821:virology*; D014057:Tomography, X-Ray Computed; D014184:Transplantation, Homologous; D016896:Treatment Outcome; D019562:Viral Load; D055815:Young Adult
chemical_list: D001215:Asparaginase