Abstract
Introduction: The designation high-grade B-cell lymphoma (HGBL) has been incorporated into the 2016 Revision of the WHO classification of lymphoid neoplasms and includes two types: (1) HGBL, not otherwise specified; and (2) HGBL with MYC and BCL2 and/or BCL6 rearrangements, also known as double or triple hit lymphoma (DHL/THL). These categories of lymphomas represent 1–2% of non-Hodgkin lymphomas and a considerable portion of DLBCL patients who are primary refractory to R-CHOP therapy. It corresponds to the designation ‘B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma’ in the 2008 WHO classification. Areas covered: This paper provides an update of HGBL, focusing on their pathologic features, prognosis, and diagnostic workup. It highlights advances in our understanding of DHL/THL. Expert commentary: The diagnosis relies on FISH testing and the major controversial question is when to perform it to diagnose virtually all DHL/THL cases, but also being cost effective. Currently there is no consensus. Considering the high refractory rate of these patients to standard R-CHOP induction, the authors recommend FISH testing in all newly diagnosed large B-cell lymphoma by using our stepwise test strategy. With the progress of molecular genetics, the prognosis will be further stratified and HGBL-NOS maybe further evolve too.
Original language | English (US) |
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Pages (from-to) | 637-648 |
Number of pages | 12 |
Journal | Expert review of hematology |
Volume | 11 |
Issue number | 8 |
DOIs | |
State | Published - Aug 3 2018 |
Keywords
- BCL2
- BCL6
- DHL
- Double hit lymphoma
- MYC
- THL
- TP53
- high grade B-cell lymphoma
- triple hit lymphoma
ASJC Scopus subject areas
- Hematology