Nationwide survey of systemic chronic active EBV infection in Japan in accordance with the new WHO classification
Open Access
- 14 July 2020
- journal article
- research article
- Published by American Society of Hematology in Blood Advances
- Vol. 4 (13), 2918-2926
- https://doi.org/10.1182/bloodadvances.2020001451
Abstract
Systemic chronic active Epstein-Barr virus infection (sCAEBV) was defined as a T- or NK-cell neoplasm in the 2017 World Health Organization (WHO) classification. To clarify the clinical features of sCAEBV under this classification and review the effects of chemotherapy, we performed a nationwide survey in Japan from 2016 through 2018 of patients with sCAEBV newly diagnosed from January 2003 through March 2016. One hundred cases were evaluated. The patients were aged 1 to 78 years (median, 21) and included 53 males and 47 females. Spontaneous regression was not observed in patients with active disease. In the childhood-onset group (age, 45 years) were female. The prognosis of the childhood-onset group was better than those of the adolescent/adult- and elderly-onset groups. The main chemotherapies used were a combination of cydosporine A, steroids, and etoposide (cooling therapy) in 52 cases and cydophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) in 45 cases. The rate of complete response (CR), defined as complete resolution of disease activity, was 17% for cooling therapy and 13% for CHOP. Virological CR was not observed. The 3-year overall survival rates in patients treated with chemotherapy only (n = 20), chemotherapy followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT; n = 47), and allo-HSCT only (n = 12) were 0%, 65%, and 82%, respectively. Distinct characteristics were observed between childhood- and elderly-onset sCAEBV, and they appeared to be different disorders. Chemotherapy is currently insufficient to resolve disease activity and eradicate infected cells. The development of an effective treatment is urgently needed.This publication has 29 references indexed in Scilit:
- Investigation of the freely available easy-to-use software ‘EZR’ for medical statisticsBone Marrow Transplantation, 2012
- Characterization of Epstein-Barr virus (EBV)-infected cells in EBV-associated hemophagocytic lymphohistiocytosis in two patients with X-linked lymphoproliferative syndrome type 1 and type 2Herpesviridae, 2012
- Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)Blood, 2011
- X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management and outcome of the diseaseBlood, 2011
- Excellent outcome of allogeneic hematopoietic SCT with reduced-intensity conditioning for the treatment of chronic active EBV infectionBone Marrow Transplantation, 2010
- Identification of Epstein‐Barr Virus (EBV)–Infected Lymphocyte Subtypes by Flow Cytometric In Situ Hybridization in EBV‐Associated Lymphoproliferative DiseasesThe Journal of Infectious Diseases, 2009
- Pathogenesis of chronic active Epstein-Barr virus infection: Is this an infectious disease, lymphoproliferative disorder, or immunodeficiency?Reviews in Medical Virology, 2006
- Proposed guidelines for diagnosing chronic active Epstein‐Barr virus infectionAmerican Journal of Hematology, 2005
- T-Cell Lymphomas Containing Epstein–Barr Viral DNA in Patients with Chronic Epstein–Barr Virus InfectionsThe New England Journal of Medicine, 1988
- The Chronic Mononucleosis SyndromeThe Journal of Infectious Diseases, 1988