A Biologic Definition of Burkitt's Lymphoma from Transcriptional and Genomic Profiling

• Published in: The New England journal of medicine Vol. 354; no. 23; pp. 2419 - 2430
• Main Authors: Hummel, Michael, Bentink, Stefan, Berger, Hilmar, Klapper, Wolfram, Wessendorf, Swen, Barth, Thomas F.E, Bernd, Heinz-Wolfram, Cogliatti, Sergio B, Dierlamm, Judith, Feller, Alfred C, Hansmann, Martin-Leo, Haralambieva, Eugenia, Harder, Lana, Hasenclever, Dirk, Kühn, Michael, Lenze, Dido, Lichter, Peter, Martin-Subero, Jose Ignacio, Möller, Peter, Müller-Hermelink, Hans-Konrad, Ott, German, Parwaresch, Reza M, Pott, Christiane, Rosenwald, Andreas, Rosolowski, Maciej, Schwaenen, Carsten, Stürzenhofecker, Benjamin, Szczepanowski, Monika, Trautmann, Heiko, Wacker, Hans-Heinrich, Spang, Rainer, Loeffler, Markus, Trümper, Lorenz, Stein, Harald, Siebert, Reiner
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 08.06.2006
• Subjects: Algorithms; Biological and medical sciences; Burkitt Lymphoma - diagnosis; Burkitt Lymphoma - genetics; Burkitt Lymphoma - pathology; Chemotherapy; Diagnosis, Differential; DNA, Neoplasm - analysis; Female; Follow-Up Studies; Gene Expression; Gene Expression Profiling; General aspects; Genes, bcl-2; Genes, Immunoglobulin; Genes, myc; Hematologic and hematopoietic diseases; Humans; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma; Lymphoma, B-Cell - classification; Lymphoma, B-Cell - diagnosis; Lymphoma, B-Cell - genetics; Lymphoma, B-Cell - mortality; Male; Medical research; Medical sciences; Middle Aged; Oligonucleotide Array Sequence Analysis; Oncogene Proteins, Fusion - genetics; Prognosis; RNA, Neoplasm - analysis; Survival Rate; Transcription, Genetic; Translocation, Genetic; Infection; Medicine; Transcription; Lymphoproliferative syndrome; Viral disease; Genomics; Burkitt lymphoma; Definition; Malignant hemopathy; Non Hodgkin lymphoma; Genome
• ISSN: 0028-4793; 1533-4406; 1533-4406
• DOI: 10.1056/NEJMoa055351

Analysis of gene-expression patterns led to the development of a molecular definition of Burkitt's lymphoma that distinguishes it from other types of mature aggressive B-cell lymphoma. Almost all cases defined by the gene-expression signature as Burkitt's lymphoma had the typical Burkitt's IG-myc translocation. Patients whose tumors did not have the Burkitt's signature had a poor outcome if the tumor cells had complex chromosomal changes. Analysis of gene-expression patterns led to the development of a molecular definition of Burkitt's lymphoma that distinguishes it from other types of mature aggressive B-cell lymphoma. Patients whose tumors did not have the Burkitt's signature had a poor outcome if the tumor cells had complex chromosomal changes. Burkitt's lymphoma and diffuse large-B-cell lymphoma are mature aggressive B-cell lymphomas. If left untreated, they follow a rapid clinical course and are fatal within months. Burkitt's lymphoma is a distinct entity that includes endemic and sporadic types and cases associated with immunodeficiency or immunosuppression. 1 With the use of chemotherapy regimens that involve methotrexate and cytarabine, cure rates for sporadic Burkitt's lymphoma approach 90 percent in children and 70 percent in adults. 2 Diffuse large-B-cell lymphoma, by contrast, is biologically and clinically heterogeneous and comprises five morphologic variants and three subtypes. 3 Treatment with a combination of chemotherapy based on cyclophosphamide, doxorubicin, vincristine, . . .