EBV-positive large B-cell lymphomas in young patients: a nodal lymphoma with evidence for a tolerogenic immune environment.
Fiche publication
Date publication
août 2015
Journal
Blood
Auteurs
Membres identifiés du Cancéropôle Est :
Dr NICOLAE Alina
Tous les auteurs :
Nicolae A, Pittaluga S, Abdullah S, Steinberg SM, Pham TA, Davies-Hill T, Xi L, Raffeld M, Jaffe ES
Lien Pubmed
Résumé
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.
Mots clés
Adolescent, Adult, Age Factors, Antineoplastic Combined Chemotherapy Protocols, therapeutic use, Child, Child, Preschool, Epstein-Barr Virus Infections, drug therapy, Female, Gene Rearrangement, B-Lymphocyte, Herpesvirus 4, Human, isolation & purification, Humans, Immune Tolerance, Immunophenotyping, Lymphoma, B-Cell, drug therapy, Lymphoma, Large B-Cell, Diffuse, drug therapy, Male, Middle Aged, Prognosis, Young Adult
Référence
Blood. 2015 Aug 13;126(7):863-72