MYC/BCL6 double‐hit lymphoma (DHL): a tumour associated with an aggressive clinical course and poor prognosis. Issue 7 (8th December 2015)

• Record Type: Journal Article
• Authors: Li, Shaoying; Desai, Parth; Lin, Pei; Yin, C Cameron; Tang, Guilin; Wang, Xuan J; Konoplev, Sergej N; Khoury, Joseph D; Bueso‐Ramos, Carlos E; Medeiros, L Jeffrey
• Is Part Of: Histopathology. Volume 68:Issue 7(2016)
• Page Start: 1090
• Page End: 1098
• Publication Date: 2015-12-08
• Subjects: BCL6/3q27 -- double‐hit lymphoma -- MYC/8q24; Histology, Pathological -- Periodicals; 611.018
• DOI: 10.1111/his.12884
• Languages: English
• ISSNs: 0309-0167

Abstract

Abstract : Aims: Large B cell lymphomas with MYC and BCL6 /3q27 rearrangements, designated MYC/BCL6 DHL, are uncommon. Our aim was to better characterize this group of tumours. Methods and results: We studied the clinicopathological features and outcome of 13 patients with MYC/BCL6 DHL and compared this group to a group of 83 MYC/BCL2 DHL patients. There were eight men and five women, with a median age of 63 years. Eleven tumours were classified as diffuse large B cell lymphomas (DLBCL) and two were B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma (BCLU). Immunohistochemical analysis showed that these tumours were positive for BCL6 (100%), BCL2 (eight of 10; 80%) and CD10 (eight of 10; 80%). Nine of 12 (75%) cases had a germinal centre B cell (GCB) immunophenotype; in one case data were incomplete. All patients were treated with chemotherapy. The clinicopathological features of MYC/BCL6 DHL were similar to MYC/BCL2 DHL, except that MYC/BCL6 DHL had a GCB immunophenotype less often. Patients with MYC/BCL6 DHL had a poor overall survival, similar to patients with MYC/BCL2 DHL ( P = 0.32). Conclusions: MYC/BCL6 DHL is an aggressive B cell lymphoma and patients often have an aggressive clinical course and poor prognosis, similar to patients with MYC/BCL2 DHL.