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Blood, 15 May 2006, Vol. 107, No. 10, pp. 4047-4052.
Prepublished online as a Blood First Edition Paper on January 19, 2006; DOI 10.1182/blood-2005-10-4213.
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NEOPLASIA
Diffuse large B-cell lymphoma in pediatric patients belongs predominantly to the germinal-center type B-cell lymphomas: a clinicopathologic analysis of cases included in the German BFM (Berlin-Frankfurt-Münster) Multicenter Trial
Ilske Oschlies,
Wolfram Klapper,
Martin Zimmermann,
Matthias Krams,
Hans-Heinrich Wacker,
Birgit Burkhardt,
Lana Harder,
Reiner Siebert,
Alfred Reiter, and
Reza Parwaresch
From the Institute of Pathology, Department of Hematopathology and Lymph Node Registry Kiel and the Institute of Human Genetics, University Hospital Schleswig-Holstein, Campus Kiel; the Department of Pediatric Hematology and Oncology, University of Hannover; and the NHL-BFM Study Center, Department of Pediatric Hematology and Oncology, Justus-Liebig-University, Giessen, Germany.
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.
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