Abstract
Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL), representing approximately 25% of diagnosed NHL. DLBCL is heterogeneous disease both clinically and genetically. The 3 most common chromosomal translocations in DLBCL involve the oncogenes BCL2, BCL6, and MYC. Double hit (DH) DLBCL is an aggressive form in which MYC rearrangement is associated with either BCL2 or BCL6 rearrangement. Patients typically present with a rapidly growing mass, often with B symptoms. Extranodal disease is often present. Though there is a paucity of prospective trials in this subtype, double hit lymphoma (DHL) has been linked to very poor outcomes when patients are treated with standard R-CHOP. There is, therefore, a lack of consensus regarding the standard treatment for DHL. Several retrospective analyses have been conducted to help guide treatment of this disease. These suggest that DA EPOCH-R may be the most promising regimen and that achievement of complete resolution predicts better long-term outcomes.
Highlights
Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous disease both in molecular pathogenesis and clinical outcome
Though immunohistochemistry (IHC) can be used to detect the over expression of MYC, BCL2, and BCL6 protein, these are not necessarily diagnostic of double hit lymphoma (DHL). Rather, when it is present by IHC in the absence of their rearrangement, detected by Fluorescence in situ hybridization (FISH), it can be classified as another entity of DLBCL called dual expresser (DE)
Petrich et al18 published a multicenter retrospective analysis looking at Double hit (DH) DLBCL patients who were treated, and compared the outcomes of those that received standard R-CHOP versus more intensive regimens (R-HYPERCVAD, DA EPOCH-R, or R-CODOX-MIVAC)
Summary
Diffuse large B-cell lymphoma (DLBCL) is a heterogeneous disease both in molecular pathogenesis and clinical outcome. Petrich et al18 published a multicenter retrospective analysis looking at DH DLBCL patients who were treated, and compared the outcomes of those that received standard R-CHOP versus more intensive regimens (R-HYPERCVAD, DA EPOCH-R, or R-CODOX-MIVAC).
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