t(14;19)/BCL3 rearrangements in lymphoproliferative disorders: A review of 23 cases

The t(14;19)(q32.3;q13.2) is a rare but recurrent translocation found in patients with B-cell malignancies, mainly in chronic B-cell lymphoproliferative disorders. When occurring in chronic lymphocytic leukemia (CLL), atypical lymphocyte morphology and immunophenotype have been reported. A high proportion of patients with CLL and t(14;19) are aged less than 40 years. t(14;19) is often associated with rapidly progressive disease, and overall prognosis is poor compared to the expected survival in chronic lymphocytic leukemia and low-grade B-cell lymphoma. t(14;19) is rarely the sole cytogenetic aberration. Trisomy 12 is the most frequent associated abnormality, and is observed in 50% of cases. t(14;19) involves the BCL3 gene, which is located at the breakpoint on chromosome 19 and is juxtaposed to the immunoglobulin heavy chain gene locus on chromosome 14 (often in the switch alpha region) in a ''head-to-head'' configuration. The translocation does not interrupt the transcriptional integrity of BCL3, but is associated with overexpression of this gene, which encodes an I kappa B-like protein and modulates the activity of the NF-kappa B transcription factors. The genes affected by overexpression of BCL3 remain to be identified. (C) Elsevier Science, Inc., 1997.