MYC/BCL2 (or MYC/BCL6) double hit lymphoma (DHL) is defined as a large B-cell lymphoma with concurrent translocations. Several studies indicate that up to 10% of diffuse large B-cell lymphomas (DLBCLs) harbouring a MYC rearrangement will have a poorer prognosis, worse still when present with a BLC2/6 rearrangement. Currently, there is a paucity of guidelines bridging together this data to decide the most effective therapy for DHL patients.
From August 2013 to October 2015, all patients with high grade B-cell NHL who had tissue samples referred to the central pathology laboratory at St. James Hospital, Dublin were included for analysis. Patient demographics, clinical features, histopathological details, systemic therapy received and patient-related outcomes were recorded from electronic or paper medical records. All cases were reviewed by a consultant haematopathologist.
152 cases of high-grade B-cell NHL were identified since FISH testing for MYC rearrangement commenced in St. James Hospital. 21 patients displayed MYC rearrangement, 9 of whom had a concurrent BCL2 rearrangement. One patient with MYC and BCL2 rearrangements also had a BCL6 rearrangement. These patients all had germinal centre cell of origin (GC COO) by Hans criteria. 4 patients with DHL had prior follicular lymphoma and 5 patients had no prior lymphoma. Patients were treated with varying regimens including standard or dose-intensified chemo-immunotherapy. All patients with DHL achieving a complete response (CR) were consolidated with transplantation (n = 5), both in first line and salvage settings. 2 patients underwent autologous transplantation and 3 underwent allogeneic transplantation. The 1-year survival for all DHL patients was 67%
In our study of all high-grade NHL, the frequency of DHL was 6%. All patients had GC COO by Hans criteria. We suggest considering referral of all patients with DLBCL for MYC-rearrangement testing at the time of initial diagnosis with consideration given to upfront dose-intensified chemotherapy and early haematogenous cell transplantation referral, once DHL status is known.
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All authors have declared no conflicts of interest.