Age-related Epstein-Barr virus (EBV)–associated B-cell lymphoproliferative disorders: comparison with EBV-positive classic Hodgkin lymphoma in elderly patients

• Published in: Blood Vol. 113; no. 12; pp. 2629 - 2636
• Main Authors: Asano, Naoko, Yamamoto, Kazuhito, Tamaru, Jun-Ichi, Oyama, Takashi, Ishida, Fumihiro, Ohshima, Koichi, Yoshino, Tadashi, Nakamura, Naoya, Mori, Shigeo, Yoshie, Osamu, Shimoyama, Yoshie, Morishima, Yasuo, Kinoshita, Tomohiro, Nakamura, Shigeo
• Format: Journal Article
• Language: English
• Published: Washington, DC Elsevier Inc 19.03.2009; Americain Society of Hematology
• Subjects: Age of Onset; Aged; Aged, 80 and over; Aging - immunology; Antigens, CD20 - analysis; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Case-Control Studies; Cocarcinogenesis; Epstein-Barr Virus Infections - epidemiology; Epstein-Barr Virus Infections - immunology; Female; Hematologic and hematopoietic diseases; Herpesvirus 4, Human - isolation & purification; Hodgkin Disease - classification; Hodgkin Disease - drug therapy; Hodgkin Disease - epidemiology; Hodgkin Disease - immunology; Hodgkin Disease - pathology; Hodgkin Disease - virology; Humans; Immunologic Surveillance; Immunophenotyping; Infectious diseases; Kaplan-Meier Estimate; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Large B-Cell, Diffuse - drug therapy; Lymphoma, Large B-Cell, Diffuse - epidemiology; Lymphoma, Large B-Cell, Diffuse - immunology; Lymphoma, Large B-Cell, Diffuse - pathology; Lymphoma, Large B-Cell, Diffuse - virology; Male; Medical sciences; Middle Aged; Necrosis; Organ Specificity; Prognosis; Proportional Hazards Models; Retrospective Studies; Sex Factors; T-Lymphocytes, Cytotoxic - immunology; Viral diseases; Gammaherpesvirinae; Human; Prognosis; Hematology; Herpesviridae; Sex; Biological marker; Hodgkin disease; Malignant hemopathy; B-Lymphocyte; Large cell lymphoma; Epstein Barr virus; Non Hodgkin lymphoma; Lymphoma; Infection; Virus; Histopathology; Age of onset; Lymphoproliferative syndrome; Viral disease; Localization; Elderly; Comparative study; Cancer
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2008-06-164806

Age-related Epstein-Barr virus–associated B-cell lymphoproliferative disorder (aEBVLPD) is a disease group characterized by EBV-associated large B-cell lymphoma in elderly without predisposing immunodeficiency. In nearly one- third of cases, aEBVLPD occurs as a polymorphous subtype with reactive cell-rich components, bearing a morphologic similarity to classic Hodgkin lymphoma (cHL). The aim of this study was to clarify clinicopathologic differences between the polymorphic subtype of aEBVLPD (n = 34) and EBV+ cHL (n = 108) in patients aged 50 years or older. Results showed that aEBVLPD was more closely associated with aggressive clinical parameters than cHL, with a higher age at onset (71 vs 63 years); lower male predominance (male-female ratio, 1.4 vs 3.3); and a higher rate of involvement of the skin (18% vs 2%), gastrointestinal tract (15% vs 4%), and lung (12% vs 2%). aEBVLPD was histopathologically characterized by a higher ratio of geographic necrosis, greater increase (> 30%) in cytotoxic T cells among background lymphocytes, higher positivity for CD20 and EBNA2, and absence of CD15 expression. As predicted by the clinical profile, aEBVLPD had a significantly poorer prognosis than EBV+ cHL (P < .001). The polymorphous subtype of aEBVLPD constitutes an aggressive group with an immune response distinct from EBV+ cHL, and requires the development of innovative therapeutic strategies.