AI Article Synopsis
- The genetics and causes of splenic marginal zone lymphoma are not well understood, particularly due to a lack of common chromosomal translocations and the mystery surrounding the significance of a 7q deletion found in about 30% of cases.
- A study identified mutations in key genes related to the NF-κB signaling pathway, specifically A20, MYD88, and CARD11, indicating potential genetic factors involved in the disease.
- The study's findings suggest that the deregulation of the TLR and BCR signaling pathways might play a crucial role in the development of splenic marginal zone lymphoma, with the mutations exhibiting a mutually exclusive pattern.
Article Abstract
The genetics and pathogenesis of splenic marginal zone lymphoma are poorly understood. The lymphoma lacks chromosome translocation, and approximately 30% of cases are featured by 7q deletion, but the gene targeted by the deletion is unknown. A recent study showed inactivation of A20, a "global" NF-κB negative regulator, in 1 of 12 splenic marginal zone lymphomas. To investigate further whether deregulation of the NF-κB pathway plays a role in the pathogenesis of splenic marginal zone lymphoma, we screened several NF-κB regulators for genetic changes by PCR and sequencing. Somatic mutations were found in A20 (6/46=13%), MYD88 (6/46=13%), CARD11 (3/34=8.8%), but not in CD79A, CD79B and ABIN1. Interestingly, these genetic changes are largely mutually exclusive from each other and MYD88 mutation was also mutually exclusive from 7q deletion. These results strongly suggest that deregulation of the TLR (toll like receptor) and BCR (B-cell receptor) signaling pathway may play an important role in the pathogenesis of splenic marginal zone lymphoma.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3347666 | PMC |
| http://dx.doi.org/10.3324/haematol.2011.054080 | DOI Listing |
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