title: Prognostic factors for transformation in asymptomatic immunoglobulin m monoclonal gammopathies.
abstract: We evaluated the risk of transformation of asymptomatic immunoglobulin (Ig) M monoclonal gammopathy (aIgM-MG) into symptomatic lymphoproliferative disease in 384 patients, in subgroups of patients with IgM MG of undetermined significance (MGUS) and smoldering Waldenstrom's macroglobulinemia (sWM). One hundred seventy-two patients with aIgM-MG with bone marrow (BM) histopathology and immunophenotyping were available for analysis. After a median of 45 months (range, 12-233 months), 45 cases of aIgM-MG (11.7%) evolved into lymphoproliferative disease, as follows: symptomatic WM (n = 41), non-Hodgkin's lymphoma (NHL; n = 2), IgM multiple myeloma (IgM-MM; n = 1), and primary amyloidosis (n = 1). Cumulative transformation probability at 5 and 10 years was 8% and 29%, respectively. The parameters significantly correlated with evolution were, at univariate analysis, BM lymphoplasmacytic infiltration, high erythrocyte sedimentation rate, hemoglobin (Hb) level, IgM size, and peripheral lymphocytosis; at multivariate analysis, the parameters were IgM size and peripheral lymphocytosis, with Hb level associated with a trend toward higher progression risk. Of the 138 cases of IgM-MGUS, 14 (10.1%) evolved (13 WM, 1 IgM-MM) after a median of 75 months (range, 12-117 months); of the 34 cases of sWM, 13 (38.2%) progressed to WM after 55 months (range, 13-154 months). In patients with IgM-MGUS, event-free survival at 5 and 10 years was 95% and 83%, respectively, and in patients with sWM, 77% and 42%, respectively (P = 0.0001). Among patients with aIgM-MG, those at high risk of evolution are patients with sWM, a distinct entity with clear BM evidence of NHL.
authors.affiliation: Division of Hematology, Niguarda Ca'Granda Hospital, Piazza Ospedale Maggiore 3, 20162 Milan, Italy. ematologia@ospedaleniguarda.it
authors.forename: Enrica
authors.initials: E
authors.lastname: Morra
authors.forename: Clara
authors.initials: C
authors.lastname: Cesana
authors.forename: Catherine
authors.initials: C
authors.lastname: Klersy
authors.forename: Luciana
authors.initials: L
authors.lastname: Barbarano
authors.forename: Sara
authors.initials: S
authors.lastname: Miqueleiz
authors.forename: Marzia
authors.initials: M
authors.lastname: Varettoni
authors.forename: Camilla
authors.initials: C
authors.lastname: Lucchesini
authors.forename: Francesca
authors.initials: F
authors.lastname: Ricci
authors.forename: Mario
authors.initials: M
authors.lastname: Lazzarino
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001853:Bone Marrow / Q000473:pathology; D002471:Cell Transformation, Neoplastic; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis*; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000276:immunology* / Q000473:pathology*; D011379:Prognosis; D012307:Risk Factors; D016019:Survival Analysis
chemical_list: D007075:Immunoglobulin M