title: Waldenström macroglobulinemia: What a hematologist needs to know.
abstract: Waldenström macroglobulinemia (WM) is a distinct hematologic malignancy characterized by a lymphoplasmacytic bone marrow infiltration and the presence of immunoglobulin (Ig)M monoclonal protein. Patients typically present at an advanced age, and a substantial proportion are asymptomatic at diagnosis. A unifying diagnosis of WM may be missed by an unsuspecting hematologist, as symptomatic patients present with a multitude of non-specific manifestations. Although constitutional and neuropathy-related symptoms predominate, concomitant IgM-induced hyperviscosity-associated features can provide useful diagnostic clues. There are specific indications for initiation of therapy. This review focuses on the most up-to-date management strategies of WM, in addition to highlighting the recent discoveries of MYD88 and CXCR4 mutations that have shed unprecedented light on the complex signaling pathways, and opened avenues for novel therapeutic targeting. Although WM remains incurable, with the rapid emergence and integration of effective novel therapies, its clinical course appears poised to improve in the foreseeable future.
authors.affiliation: Division of Hematology Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA; Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address: kapoor.prashant@mayo.edu.
authors.forename: Prashant
authors.initials: P
authors.lastname: Kapoor
authors.affiliation: Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address: paludo.jonas@mayo.edu.
authors.forename: Jonas
authors.initials: J
authors.lastname: Paludo
authors.affiliation: Division of Hematology Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address: vallumsetla.nishanth@mayo.edu.
authors.forename: Nishanth
authors.initials: N
authors.lastname: Vallumsetla
authors.affiliation: Division of Hematology Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA; Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. Electronic address: greipp.philip@mayo.edu.
authors.forename: Philip R
authors.initials: PR
authors.lastname: Greipp
mesh_terms: D006801:Humans; D008258:Waldenstrom Macroglobulinemia
keywords: IgM monoclonal gammopathy; Indolent lymphoma; Lymphoplasmacytic lymphoma