Abstract
Diffuse large B-cell lymphoma (DLBCL) in adults is a heterogeneous disease. Biologic subgroups of DLBCL with a favorable prognosis (germinal center B-cell-like, GCB) and with a poor prognosis (activated B-cell-like, ABC) have been defined by gene expression profiling and can be distinguished by immunohistochemistry. In contrast to their adult counterparts, children with DLBCL have an excellent prognosis. We analyzed 63 cases of DLBCL in pediatric patients by immunohistochemistry and fluorescence in situ hybridization (FISH) and found a striking predominance of a GCB subtype, which might explain the good clinical outcome in these lymphomas. Interestingly, FISH applied to 50 of these cases, as well as conventional cytogenetics available in 3 cases, revealed absence of the translocation t(14;18) involving the BCL2 gene, which is present in about 15% of adult GCB subtype DLBCL. Our data indicate that pediatric DLBCL differs from adult DLBCL and might comprise a biologically unique subgroup of DLBCL from which important insights into the pathogenesis and biology of this disease might be gained.
Highlights
Introduction cases ofDiffuse large B-cell lymphoma (DLBCL) in pediatric patients by immunohistochemistry and fluorescence in situ hybridization (FISH) and found a striking predominance of a germinal center B-cell type (GCB) subtype, which might explain the good clinical outcome in these lymphomas
The germinal center B-cell type (GCB) is characterized by expression of genes found in normal germinal center B cells and shows favorable outcome compared to the activated B-cell type (ABC) and type 3 DLBCL
The study population can be considered representative for all nonlymphoblastic, nonBurkitt B-cell non-Hodgkin lymphoma occurring in children in Germany
Summary
The duration of event-free survival (EFS) is defined as the time from diagnosis until the date of the first adverse event (tumor failure, death of any cause, or the development of a second malignancy), or if no such event occurred, until the date of latest contact. The statistical analysis was carried out using SAS (SAS-PC, Version 9.1; SAS Institute, Cary, NC)
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