Journal Article

T/NK cell type chronic active Epstein–Barr virus disease in adults: an underlying condition for Epstein–Barr virus-associated T/NK-cell lymphoma

Ito, Yoshinori, Isobe, Yasushi, Sugimoto, Koichi, Hamano, Yasuharu, Komatsu, Norio, Setoguchi, Yasuhiro, Kimura, Hiroshi, Aritaka, Nanae

2012

Overview

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.

• Publisher: BMJ Publishing Group Ltd and Association of Clinical Pathologists,BMJ Publishing Group LTD
• Subject: Adult / Allogeneic haematopoietic cell transplantation / Antigens / Antineoplastic Combined Chemotherapy Protocols - therapeutic use / Asparaginase - administration & dosage / Biopsy / Blood / Blotting, Southern / Chemotherapy / chronic active EBV disease / Chronic Disease / Deoxyribonucleic acid / DNA / EBV / Epstein-Barr virus / Epstein-Barr Virus Infections - complications / Epstein-Barr Virus Infections - diagnosis / Epstein-Barr Virus Infections - immunology / Epstein-Barr Virus Infections - therapy / Epstein-Barr Virus Infections - virology / Female / Fever / genetics / Hematopoietic Stem Cell Transplantation / Hepatitis / Humans / Immunohistochemistry / Infections / Killer Cells, Natural - immunology / Killer Cells, Natural - virology / l-asparaginase / leukaemia / Liver / lung / lung cancer / Lymphatic system / Lymphocytes / Lymphoma / Lymphoma, Extranodal NK-T-Cell - diagnosis / Lymphoma, Extranodal NK-T-Cell - immunology / Lymphoma, Extranodal NK-T-Cell - therapy / Lymphoma, Extranodal NK-T-Cell - virology / Male / Medical prognosis / Middle Aged / T-Lymphocytes - immunology / T-Lymphocytes - virology / T/NK cell type / Tomography, X-Ray Computed / Transplantation, Homologous / Transplants & implants / Treatment Outcome / Viral Load / Young Adult