title: Waldenström macroglobulinemia: from biology to treatment.
abstract: Waldenström macroglobulinemia (WM) is distinct B-cell lymphoproliferative disorder primarily characterized by bone marrow infiltration of lymphoplasmacytic cells along with production of a serum monoclonal (IgM). In this review, we describe the biology of WM, the diagnostic evaluation for WM with a discussion of other conditions that are in the differential diagnosis and clinical manifestations of the disease as well as current treatment options. Within the novel agents discussed are everolimus, perifosine, enzastaurin, panobinostat, bortezomib and carfilzomib, pomalidomide and ibrutinib. Many of the novel agents have shown good responses and have a better toxicity profile compared to traditional chemotherapeutic agents, which makes them good candidates to be used as primary therapies for WM in the future.
authors.affiliation: Department of Medical Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA.
authors.forename: Ilyas
authors.initials: I
authors.lastname: Sahin
authors.forename: Houry
authors.initials: H
authors.lastname: Leblebjian
authors.forename: Steven P
authors.initials: SP
authors.lastname: Treon
authors.forename: Irene M
authors.initials: IM
authors.lastname: Ghobrial
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D002869:Chromosome Aberrations; D020869:Gene Expression Profiling; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D007155:Immunologic Factors / Q000627:therapeutic use; D061988:Proteasome Inhibitors / Q000627:therapeutic use; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D040901:Proteomics; D000069283:Rituximab; D008258:Waldenstrom Macroglobulinemia / Q000378:metabolism* / Q000473:pathology / Q000628:therapy
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007075:Immunoglobulin M; D007155:Immunologic Factors; D061988:Proteasome Inhibitors; D047428:Protein Kinase Inhibitors; D000069283:Rituximab