Genetics and Prognostication in Splenic Marginal Zone Lymphoma:

A Peculiar Case of Splenic Marginal Zone Lymphoma and Review of Literature

Splenic marginal zone lymphoma: from genetics to management.

Splenic marginal zone lymphoma (SMZL) is a rare B-cell malignancy involving the spleen, bone marrow, and frequently the blood. SMZL lymphomagenesis involves antigen and/or superantigen stimulation and molecular deregulation of genes (NOTCH2 and KLF2) involved in the physiological differentiation of spleen marginal zone B cells. Diagnosis requires either spleen histology or, alternatively, the d...

Cotargeting BCL-2 and BCL-XL for maximal efficacy in ALL.

1. Spina V, Khiabanian H, Messina M, et al. The genetics of nodal marginal zone lymphoma. Blood. 2016; 128(10):1362-1373. 2. Thieblemont C, Molina T, Davi F. Optimizing therapy for nodal marginal zone lymphoma. Blood. 2016; 127(17):2064-2071. 3. Kanellis G, Roncador G, Arribas A, et al. Identification of MNDA as a new marker for nodal marginal zone lymphoma. Leukemia. 2009;23(10): 1847-1857. 4....

BCR and TLR signaling pathways are recurrently targeted by genetic changes in splenic marginal zone lymphomas.

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 ...

Genetics and Prognostication in Splenic Marginal Zone Lymphoma: Revelations from Deep Sequencing.

PURPOSE Mounting evidence supports the clinical significance of gene mutations and immunogenetic features in common mature B-cell malignancies. EXPERIMENTAL DESIGN We undertook a detailed characterization of the genetic background of splenic marginal zone lymphoma (SMZL), using targeted resequencing and explored potential clinical implications in a multinational cohort of 175 patients with SM...