Epstein-Barr virus–positive diffuse large B-cell lymphoma in elderly patients is rare in Western populations

• Published in: Human pathology 2010-03, Vol.41 (3), p.352-357
• Main Authors: Hoeller, Sylvia, MD, Tzankov, Alexandar, MD, Pileri, Stefano A., MD, Went, Philip, MD, Dirnhofer, Stephan, MD
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Biological and medical sciences; Child; Diffuse large b-cell lymphoma; EBV; EBV-positive diffuse large b-cell lymphoma of the elderly; Epstein barr virus; Epstein-Barr Virus Infections - epidemiology; Epstein-Barr Virus Infections - pathology; Epstein-Barr Virus Infections - virology; European Continental Ancestry Group; Female; Hematologic and hematopoietic diseases; Herpesvirus 4, Human; Humans; Immunohistochemistry; In Situ Hybridization; Incidence; Infectious diseases; Investigative techniques, diagnostic techniques (general aspects); Kaplan-Meier Estimate; Ki-1 Antigen; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, Large B-Cell, Diffuse - epidemiology; Lymphoma, Large B-Cell, Diffuse - pathology; Lymphoma, Large B-Cell, Diffuse - virology; Male; Medical sciences; Middle Aged; Necrosis - pathology; Necrosis - virology; Pathology; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Patient Selection; Prognosis; RNA, Viral; Tissue Array Analysis; Viral diseases
• ISSN: 0046-8177; 1532-8392
• DOI: 10.1016/j.humpath.2009.07.024

Summary In the currently published World Health Organization-Classification, the new entity of Epstein-Barr virus–positive diffuse large B-cell lymphoma of the elderly was introduced largely based on findings from East-Asian populations. Little is known about its frequency or characteristics in the West, especially in European populations. Using a tissue microarray approach, we identified 8 out of 258 diffuse large B-cell lymphoma cases fulfilling the World Health Organization criteria of an Epstein-Barr virus–positive diffuse large B-cell lymphoma of the elderly, suggesting an incidence of 3.1% in a European population. The median patient age was 65 years. The highest diagnostic sensitivity was only achieved by EBER in situ hybridization. No correlation between Epstein-Barr virus status and outcome was noted except in latency type 3 lymphomas, which had a very poor survival. Sixty-seven percent of Epstein-Barr virus–positive cases showed the presence of necrosis and 50% expressed the activation marker CD30. However, no morphological or immunohistochemical features reliably distinguished all Epstein-Barr virus–positive diffuse large B-cell lymphoma cases. Thus, to identify these Epstein-Barr virus–positive diffuse large B-cell lymphoma in the elderly, EBER in situ hybridization of all de novo diffuse large B-cell lymphoma cases of patients older than 50 years should be considered. In summary, Epstein-Barr virus–positive diffuse large B-cell lymphoma of the elderly is rare in Europeans older than 50 years. It can only be diagnosed by EBER-ISH, and its precise prognostic role is unclear. Whether routine testing of all diffuse large B-cell lymphoma patients older than 50 years can be recommended depends essentially on its clinical relevance. Future studies are needed to address this question.