Splenic marginal-zone lymphoma: ontogeny and genetics

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Authors

BALIAKAS Panagiotis STREFFORD Jonathan C. BIKOS Vasileios PARRY Marina STAMATOPOULOS Kostas OSCIER David

Year of publication 2015
Type Article in Periodical
Magazine / Source Leukemia & Lymphoma
MU Faculty or unit

Central European Institute of Technology

Citation
Web http://informahealthcare.com/doi/abs/10.3109/10428194.2014.919636
Doi http://dx.doi.org/10.3109/10428194.2014.919636
Field Oncology and hematology
Keywords Lymphoma and Hodgkin disease; cytogenetics; molecular genetics
Attached files
Description Splenic marginal-zone lymphoma (SMZL) is a rare tumor that has recently emerged as a prototype for how the interplay between genetics and environment shapes the natural history of lymphomas. Indeed, the recent identification of molecular immunogenetic subgroups within SMZL may prove to be relevant not only for the sub-classification of the disease but also for improved understanding of the underlying biology. In contrast to other B-cell lymphomas, SMZL lacks a characteristic genetic lesion, although the majority of cases harbor genomic aberrations, as recently revealed by high-throughput studies that identified recurrent genetic aberrations, several in pathways related to marginal-zone differentiation and B-cell signaling. Here we provide an overview of recent research into the molecular and cellular biology of SMZL and related disorders, with special emphasis on immunogenetics and genomic aberrations, and discuss the value of molecular and cellular markers for the diagnosis and differential diagnosis of these entities.
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