title: Progression in smoldering Waldenstrom macroglobulinemia: long-term results.
abstract: The purpose of this study was to define the risk of progression and survival of patients with smoldering Waldenström macroglobulinemia (SWM). SWM is defined clinically as having a serum monoclonal IgM protein≥3 g/dL and/or≥10% bone marrow lymphoplasmacytic infiltration but no evidence of end-organ damage (anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly). We searched a computerized database and reviewed the medical records of all patients at Mayo Clinic who fulfilled the criteria of SWM between 1974 and 1995. During 285 cumulative person-years of follow-up of the 48 patients with SWM (median, 15.4 years), 34 (71%) progressed to symptomatic Waldenström macroglobulinemia (WM) requiring treatment, one to primary amyloidosis, and one to lymphoma (total, 75%). The cumulative probability of progression to symptomatic WM, amyloidosis, or lymphoma was 6% at 1 year, 39% at 3 years, 59% at 5 years, and 68% at 10 years. The major risk factors for progression were percentage of lymphoplasmacytic cells in the bone marrow, size of the serum M-spike, and the hemoglobin value. Patients with SWM should be followed and not treated until symptomatic WM develops. Treatment on a clinical trial for those at greatest risk of progression should be considered.
authors.affiliation: Division of Hematology, College of Medicine, Mayo Clinic, Rochester, MN 55905, USA. kyle.robert@mayo.edu
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
authors.forename: Joanne T
authors.initials: JT
authors.lastname: Benson
authors.forename: Dirk R
authors.initials: DR
authors.lastname: Larson
authors.forename: Terry M
authors.initials: TM
authors.lastname: Therneau
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.forename: Shaji
authors.initials: S
authors.lastname: Kumar
authors.forename: L Joseph
authors.initials: LJ
authors.lastname: Melton
authors.forename: S Vincent
authors.initials: SV
authors.lastname: Rajkumar
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000453:epidemiology / Q000401:mortality / Q000473:pathology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008223:Lymphoma / Q000453:epidemiology / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D016019:Survival Analysis; D008258:Waldenstrom Macroglobulinemia / Q000453:epidemiology / Q000401:mortality / Q000473:pathology* / Q000628:therapy