title: Waldenström macroglobulinemia.
abstract: Waldenström macroglobulinemia (WM) is an IgM-secreting B-cell lymphoproliferative disorder, with strong familial predisposition. MYD88 L265P and CXCR4 WHIM mutations are common in WM and support the growth and survival of WM cells. Clinical manifestations of disease are related to both tumor cell infiltration and paraprotein production. Current treatment includes monoclonal antibodies, alkylating agents, nucleoside analogs, proteasome inhibitors, immunomodulatory drugs, and signal inhibitors. Short- and long-term toxicities should be weighed in treatment decisions with use of these agents. Elucidation of the signaling pathways involved in WM is helping to advance targeted therapeutics for WM and includes efforts directed at MYD88 and CXCR4 signaling.
authors.affiliation: Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA 02215, USA. Electronic address: steven_treon@dfci.harvard.edu.
authors.forename: Steven P
authors.initials: SP
authors.lastname: Treon
authors.affiliation: Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA 02215, USA.
authors.forename: Zachary R
authors.initials: ZR
authors.lastname: Hunter
authors.affiliation: Bing Center for Waldenström's Macroglobulinemia, Dana-Farber Cancer Institute, Harvard Medical School, 450 Brookline Avenue, Boston, MA 02215, USA.
authors.forename: Jorge J
authors.initials: JJ
authors.lastname: Castillo
authors.affiliation: Department of Molecular Medicine, Amyloidosis Research and Treatment Center, University Hospital Policlinico San Matteo, Viale C. Golgi, 19, Pavia 27100, Italy.
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
mesh_terms: D001402:B-Lymphocytes / Q000378:metabolism / Q000473:pathology*; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000473:pathology*; D008957:Models, Genetic; D009154:Mutation; D053594:Myeloid Differentiation Factor 88 / Q000235:genetics; D019718:Receptors, CXCR4 / Q000235:genetics; D015398:Signal Transduction / Q000235:genetics; D008258:Waldenstrom Macroglobulinemia / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*
chemical_list: C527276:CXCR4 protein, human; C507779:MYD88 protein, human; D053594:Myeloid Differentiation Factor 88; D019718:Receptors, CXCR4
keywords: CXCR4 WHIM mutations; Lymphoproliferative disorder; MYD88 L265P; Morbidity; Treatment options; Waldenström macroglobulinemia