MYD88 mutation and Von Willebrand’s – pro
The MYD88 L265P mutation has been recently identified in >90% of bone-marrow-based lymphoplasmacytic lymphoma, and can help make that diagnosis. There is a low incidence of L265P MYD88 mutation in other systemic CD5-negative B-cell lymphoproliferative disorders including atypical chronic lymphocytic leukemia, nodal marginal zone lymphoma (MZL), splenic MZL and mucosa-associated lymphoid tissue (MALT)-type MZL. A recent report used MYD88 negativity to help establish a diagnosis of nonsecretory IgM multiple myeloma from WM in a patient that secreted only free light chains but who had cytoplasmic IgM. The MYD88 status is now known to influence the overall prognosis as well as response to targeted therapies such as ibrutinib in patients with LPL/WM [7, 8]. Specifically, the efficacy of ibrutinib in previously treated LPL/WM patients revealed “major responses” with response rates highest among patients with MYD88 L265P mutation and wild-type CXCR4 suggesting
Hamadeh F et al, MYD88 L265P mutation analysis helps define nodal lymphoplasmacytic lymphoma.Send toMod Pathol. 2015 Apr;28(4):564-74
Treon S. P., Xu L., Yang G., et al. MYD88 L265P somatic mutation in Waldenström’s macroglobulinemia. New England Journal of Medicine. 2012;367(9):826–833. doi: 10.1056/NEJMoa1200710.
Xu L., Hunter Z. R., Yang G., et al. MYD88 L265P in Waldenström macroglobulinemia, immunoglobulin M monoclonal gammopathy, and other B-cell lymphoproliferative disorders using conventional and quantitative allele-specific polymerase chain reaction. Blood. 2013;121(11):2051–2058. doi: 10.1182/blood-2012-09-454355.
Jiménez C., Sebastián E., Chillón M. C., et al. MYD88 L265P is a marker highly characteristic of, but not restricted to, Waldenström’s macroglobulinemia. Leukemia. 2013;27(8):1722–1728. doi: 10.1038/leu.2013.62
Treon S. P., Tripsas C. K., Meid K., et al. Ibrutinib in previously treated Waldenström’s macroglobulinemia. New England Journal of Medicine. 2015;372(15):1430–1440.
Treon S. P., Xu L., Hunter Z. MYD88 mutations and response to ibrutinib in Waldenström’s Macroglobulinemia. New England Journal of Medicine. 2015;373(6):584–586.
Treon S. P., Hunter Z. R. A new era for Waldenstrom macroglobulinemia: MYD88 L265P. Blood. 2013;121(22):4434–4436. doi: 10.1182/blood-2013-04-494849.