BCL-6 AND LYMPHOPROLIFERATIVE DISORDERS

Citation
Gy. Michaud et al., BCL-6 AND LYMPHOPROLIFERATIVE DISORDERS, Leukemia & lymphoma, 26(5-6), 1997, pp. 515-525
Citations number
38
Categorie Soggetti
Hematology
Journal title
ISSN journal
10428194
Volume
26
Issue
5-6
Year of publication
1997
Pages
515 - 525
Database
ISI
SICI code
1042-8194(1997)26:5-6<515:BALD>2.0.ZU;2-S
Abstract
We report a series of 37 cases of lymphoproliferative disorders with 3 q27 structural chromosomal abnormalities. Breakpoints at 3q27, the sit e of the bcl-6 gene, appear in a broad range of B cell lymphoma histol ogies but are most frequently detected in follicular lymphomas lacking a t(14;18) and diffuse large cell lymphomas. The majority of 3q27 rea rrangements result from translocations involving the immunoglobulin he avy or light chain genes, however, involvement of other partner chromo somes is also observed. Molecular rearrangement of bcl-6 is demonstrab le in a subset of cases. Bcl-6 is a recently identified gene encoding a zinc-finger protein. it is normally expressed in germinal center B c ells where it is believed to have a developmental or differentiation f unction. Transcriptional deregulation of bcl-6 through translocations, submicroscopic molecular rearrangements or point mutations may be res ponsible for this gene's putative lymphomagenic potential.