The gray zone between Burkitt's lymphoma and diffuse large B-cell lymphoma from a genetics perspective

Abstract

It has long been recognized that the border between classical Burkitt's lymphoma (BL) and classical diffuse large B-cell lymphoma (DLBCL) is hard to determine. Instead, both classical lymphoma entities seem to be the extreme ends of a spectrum of diseases that contains a group of lymphomas characterized predominately by the fact that they are hard to assign to the one or the other group. This gray zone has been recently termed "lymphoma, unclassifiable, with features intermediate between DLBCL and BL" by the updated WHO classification. The term "intermediate" resembles that from a recent gene-expression study of mature aggressive B-cell lymphomas, although, notably, it is used differently. Intermediate lymphomas according to the WHO classification clearly are a temporary container of different biologic subtypes of aggressive lymphoma, from which several might be associated with an unfavorable clinical outcome. The present review aims at describing the morphologic, clinical, and biologic heterogeneity of the intermediate lymphomas and, moreover, attempts to propose testable subgroups based on age and presence of genetic aberrations.