title: Genomic abnormalities of Waldenström macroglobulinemia and related low-grade B-cell lymphomas.
abstract: Waldenström macroglobulinemia (WM) is a lymphoproliferative disease characterized by a heterogeneous lymphoplasmacytic bone marrow infiltrate and monoclonal immunoglobulin M production. WM shows similarities in presentations with related B-cell malignancies, sometimes making it difficult to distinguish them. To better characterize the genetic basis of WM, we performed a comparative genomic analysis with the related entities, lymphoplasmacytic lymphomas without monoclonal immunoglobulin M protein, marginal zone lymphomas, chronic lymphocytic leukemia, and monoclonal gammopathy of undetermined significance. Overall, WM shows a very stable karyotype and shares most of the chromosomal abnormalities with most of the indolent B-cell malignancies. Trisomy 4 is unique to WM; however, no candidate genes have been identified in the chromosome. Abnormalities that affect myeloid differentiation primary response 88 (MYD88)--interleukin-1 receptor-associated kinase 4 (IRAK4) and nuclear factor kappa B (NF-κB) signaling pathways were found in a significant proportion of WM cases, which suggest their relevance in the pathogenesis of the disease and opening new avenues that may be a guide to design novel therapeutic approaches.
authors.affiliation: Department of Hematology, Oncology, Mayo Clinic, Scottsdale, AZ 85259-5494, USA.
authors.forename: Esteban
authors.initials: E
authors.lastname: Braggio
authors.forename: Rafael
authors.initials: R
authors.lastname: Fonseca
mesh_terms: D002869:Chromosome Aberrations; D055028:Comparative Genomic Hybridization; D056915:DNA Copy Number Variations; D055106:Genome-Wide Association Study; D023281:Genomics; D006801:Humans; D016393:Lymphoma, B-Cell / Q000235:genetics*; D008258:Waldenstrom Macroglobulinemia / Q000235:genetics*