title: Waldenstrom Macroglobulinemia: Genomic Aberrations and Treatment.
abstract: Waldenström macroglobulinemia (WM) is a rare, indolent, and monoclonal immunoglobulin M-associated lymphoplasmacytic disorder with unique clinicopathologic characteristics. Over the past decade, remarkable progress has occurred on both the diagnostic and therapeutic fronts in WM. A deeper understanding of the disease biology emanates from the seminal discoveries of myeloid differentiation primary response 88 (MYD88) L265P somatic mutation in the vast majority of cases and C-X-C chemokine receptor, type 4, mutations in about a third of patients. Although WM remains an incurable malignancy, and the indications to initiate treatment are largely unchanged, the therapeutic armamentarium continues to expand. Acknowledging the paucity of high-level evidence from large randomized controlled trials, herein, we evaluate the genomic aberrations and provide a strategic framework for the management in the frontline as well as the relapsed/refractory settings of symptomatic WM.
authors.affiliation: Mayo Clinic, Scottsdale, AZ, USA.
authors.forename: Prashant
authors.initials: P
authors.lastname: Kapoor
authors.affiliation: Mayo Clinic, Scottsdale, AZ, USA.
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Ansell
authors.affiliation: Mayo Clinic, Scottsdale, AZ, USA. Braggio.Esteban@mayo.edu.
authors.forename: Esteban
authors.initials: E
authors.lastname: Braggio
mesh_terms: D002869:Chromosome Aberrations; D006801:Humans; D008258:Waldenstrom Macroglobulinemia / Q000235:genetics* / Q000628:therapy*
keywords: BTK inhibitors; CXCR4; IgM; MYD88; Waldenstrom’s macroglobulinemia