Title : High grade B-cell lymphoma with MYC, BCL2 w/wo BCL6 rearrangements
Abstract:
High grade B-cell lymphoma with MYC and BCL2 with or without BCL6 rearrangements is also called double/triple hit lymphoma (DHL). MYC and BCL6 rearrangements, the previous MYC and BCL6 DHL has been excluded from this category in the 5th WHO classification. In the past few years, numerous case series of MYC/BCL2 DHL have been reported in the literature. Most cases of MYC/BCL2 DHL morphologically resemble diffuse large B-cell lymphoma (DLBCL) or high grade B-cell lymphoma, not otherwise specified (previous name in 2008 WHO: B cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma). These tumors have a germinal center B-cell immunophenotype but an aggressive clinical course characterized by a high proliferation rate, advanced-stage disease, extranodal involvement, high International Prognostic Index score and high serum lactate dehydrogenase levels. All tumors have a complex karyotype. Despite a variety of therapeutic approaches that have been used to date, patients with DHL have a poor prognosis. Here we will discuss the clinicopathologic, immunophenotypic, cytogenetic, and prognostic features of DHL and some remaining issues.