Mediastinal B-cell lymphoma with MYC, BCL2, and BCL6 rearrangements

AbstractPrimary mediastinal B-cell lymphoma (PMBL) is a rare but aggressive form of non-Hodgkin lymphoma (NHL) that shares significant overlap with Diffuse large B-cell lymphoma (DLBCL). Double- (DH) and triple-hit (TH) lymphomas are aggressive B-cell lymphomas with translocations involvingMYC + BCL2 or BCL6 andMYC + BCL2 + BCL6, respectively. There are only 2 previous reports of DH in PMBL and no reports of TH. Here, we present the case of a 23-year-old female with post-treatment relapse of PMBL into a PMBL-TH, after initially presenting withMYC but without eitherBCL2 orBCL6 rearrangement. Immunohistochemistry (IHC), fluorescence in situ hybridization (FISH), and the Lymph3Cx gene expression assay were used to confirm the diagnosis. This case initiates the literature for PMBL-TH and expands the overall knowledge base regarding DH/TH in B-cell lymphomas. It also further emphasizes the utility of genetic and molecular assays in clinical identification of unique B-cell lymphoma  subtypes when considering potential treatment courses.
Source: Journal of Hematopathology - Category: Pathology Source Type: research