MYC/BCL6 double-hit lymphoma (DHL): A tumour associated with an aggressive clinical course and poor prognosis

Shaoying Li, Parth Desai, Pei Lin, C. Cameron Yin, Guilin Tang, Xuan J. Wang, Sergej N. Konoplev, Joseph D. Khoury, Carlos E. Bueso-Ramos, L. Jeffrey Medeiros

Research output: Contribution to journalArticlepeer-review

80 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1090-1098
Number of pages9
JournalHistopathology
Volume68
Issue number7
DOIs
StatePublished - Jun 1 2016
Externally publishedYes

Keywords

  • BCL6/3q27
  • Double-hit lymphoma
  • MYC/8q24

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology

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