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Original Article

Molecular Pathways in Low Grade B-Cell Lymphoma

, , , &
Pages 107-113 | Received 07 May 1996, Published online: 01 Jul 2009
 

Abstract

Low grade B-cell non-Hodgkin's lymphomas (B-NHL) represent a markedly heterogeneous group of lymphoproliferative disorders, including B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CCL/SLL), lymphoplasmacytoid lymphoma (LPL), follicular lymphoma (FL), mucosa-associated lymphoid tissue lymphoma (MALTL), and splenic lymphoma with villous lymphocytes (SLVL). The molecular pathogenesis of low grade B-NHL is characterized by distinct genetic pathways which selectively associate with each clinico-pathologic category. At diagnosis, B-CLL/SLL frequently display deletions of 13q14 and tri-somy 12, whereas evolution to Richter's syndrome associates with disruption of p.53. LPL carries t(9;14)(p13;q32) in 40–50% of the cases, leading to the deregulated expression of the PAX-5 gene. FL consistently harbors rearrangements of BCL-2 independent of the cytologic variant. With time, a fraction of FL cases accumulates mutations of p.53 and evolves into a high grade B-NHL. Low grade MALTL are characterized by the frequent occurrence of tri-somy 3 and, occasionally, by p53 mutations. SLVL cames p.53 mutations in a fraction of cases. The identification of distinct genetic categories among low grade B-NHL may help in the therapeutic stratification of these disorders. In addition, genetic lesions of low grade B-NHL have proved to be a useful molecular marker for monitoring minimal residual disease.

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