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Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin’s lymphoma. While many patients will be cured with Rituximab-CHOP, 30%–50% will either relapse or have disease refractory to treatment.1 The International Prognostic Index is not sufficiently accurate to stratify these patients into prognostic subgroups, thus gene expression profiling (GEP) and the identification of gene rearrangements (R) involving Myc, Bcl-2 and Bcl-6 are instead used for risk stratification. GEP or more commonly immunohistochemistry (IHC)-based algorithms are used to stratify patients according to their cell of origin (COO), broadly dividing them into germinal centre B (GCB), and non-GCB subgroups. The latter group incorporates the activated B-cell (ABC) subtype, which is associated with significantly worse outcomes when treated with standard chemotherapy regimens, as compared with the GCB subgroup.2
Double hit (DH) lymphomas are characterised by the presence of a Myc gene R with a concurrent Bcl-2 or Bcl-6 gene R, while triple hit (TH) lymphomas have all three. The rearrangement status of these lymphomas is reflected in the updated WHO classification of lymphoid neoplasms in 2017. DH/TH lymphomas account for 5%–10% of (morphologic) DLBCL.2 Interestingly, 80% of DH lymphomas have concurrent Myc and Bcl-2 R and are associated with the GCB subtype, while dual translocations of Myc and Bcl-6 are more commonly linked to the ABC phenotype.3
Overexpression of c-myc protein (defined as >40% positive neoplastic …
Handling editor Mary Frances McMullin.
Contributors KD, DMT and KO’H performed the audit. KD carried out the statistical tests. KD drafted the paper. RF, MJ, EV and DMT were responsible for critical revision of the paper. JW, GC and YC performed the tests.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
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