EBV-positive large B-cell lymphomas in young patients: a nodal lymphoma with evidence for a tolerogenic immune environment.

Nicolae, Alina; Pittaluga, Stefania; Abdullah, Shahed; Steinberg, Seth M; Pham, Thu Anh; Davies-Hill, Theresa; Xi, Liqiang; Raffeld, Mark; Jaffe, Elaine S (2015). EBV-positive large B-cell lymphomas in young patients: a nodal lymphoma with evidence for a tolerogenic immune environment. Blood, 126(7), pp. 863-872. American Society of Hematology 10.1182/blood-2015-02-630632

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Few studies have reported Epstein-Barr virus-positive (EBV(+)) large B-cell lymphomas (LBCLs) in young patients without immunodeficiency. We identified 46 such cases in patients ≤45 years of age and analyzed the clinical and pathological characteristics. EBV(+) LBCLs affected predominantly males (male:female = 3.6:1), with a median age of 23 years (range, 4-45 years). All patients presented with lymphadenopathy and 11% also had extranodal disease. Morphologically, 3 patterns were identified: T-cell/histiocyte-rich large B-cell lymphoma-like (n = 36), gray zone lymphoma (n = 7), and diffuse LBCL-not otherwise specified (n = 3). Tumor cells (EBV(+) in >90% of cells) expressed B-cell antigens, were often CD30 and PD-L1 positive, and showed a nongerminal center immunophenotype. A total of 93% expressed EBV latency type II and 7% latency type III. Indoleamine 2,3-dioxygenase was expressed on background accessory cells. The most common treatment regimen was rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (58%), with local radiation therapy added in 21%. With a median follow-up of 22 months, 82% of patients are in clinical remission and only 8% died of disease. Younger patients achieved a significantly higher overall survival than prior series of EBV(+) LBCLs reported in the elderly (P < .0001). In conclusion, EBV(+) LBCLs are not restricted to the elderly. Young patients present with nodal disease and have a good prognosis.

Item Type:	Journal Article (Original Article)
Division/Institute:	04 Faculty of Medicine > Service Sector > Institute of Pathology
UniBE Contributor:	Nicolae, Alina
Subjects:	500 Science > 570 Life sciences; biology 600 Technology > 610 Medicine & health
ISSN:	0006-4971
Publisher:	American Society of Hematology
Language:	English
Submitter:	Ekkehard Hewer
Date Deposited:	20 Apr 2017 14:46
Last Modified:	05 Dec 2022 15:03
Publisher DOI:	10.1182/blood-2015-02-630632
PubMed ID:	25999451
URI:	https://boris.unibe.ch/id/eprint/95943