title: Clinical characteristics and outcome of immunoglobulin M-related disorders.
abstract: We analyzed the clinical features and prognostic factors for transformation of immunoglobulin Mrelated disorders (IgM-RDs) to malignant lymphoproliferative disease (MLD) in 83 patients with IgM-RDs. We studied 19 patients with type I cryoglobulinemias, 56 patients with type II cryoglobulinemias, 5 patients with peripheral neuropathies (PNs), and 3 patients with idiopathic thrombocytopenic purpuras. Fourteen patients with cryoglobulinemias presented with mild to moderate hepatomegaly with or without splenomegaly. Fourteen patients with type II cryoglobulinemias had arthralgias and/or vascular purpura (12 receiving corticosteroids), and 7 presented with PN. These latter patients and those with PNs without cryoglobulinemia were treated with steroids, cyclophosphamide, or polychemotherapy with/without plasma-exchange. Cumulative probability of evolution to MLD at 5 years was 15% (95% CI; 5%-25%). At a median of 62 months (12-195 months), 8 cases of IgM-RDs (8.4%) evolved to overt Waldenstrom's macroglobulinemia (n = 6), 1 case to non-Hodgkin's lymphoma, and 1 case to B-cell chronic lymphocytic leukemia. At univariate analysis, male sex (P = 0.02), IgM level > or = 3 g/dL (P < 0.0001), detectable Bence Jones proteinuria (P = 0.0005), lymphocytosis (P = 0.049), and high erythrocyte sedimentation rate (P = 0.003) significantly correlated with the evolution risk. Age, blood cell counts, b2-microglobulin level, degree of marrow lymphoplasmacytic infiltration, type of cryoglobulinemia, and hepatitis C virus positivity did not correlate with transformation. Although IgM-RDs represent a distinct clinical entity frequently requiring treatment in view of the IgM-related symptoms, their evolution probability and prognostic factors for malignant transformation seem to widely overlap those described for asymptomatic IgM monoclonal gammopathies.
authors.affiliation: Department of Hematology, Niguarda Ca'Granda Hospital, Piazza Ospedale Maggiore 3, 20162 Milan, Italy. clara.cesana@aovimercate.org
authors.forename: Clara
authors.initials: C
authors.lastname: Cesana
authors.forename: Luciana
authors.initials: L
authors.lastname: Barbarano
authors.forename: Sara
authors.initials: S
authors.lastname: Miqueleiz
authors.forename: Camilla
authors.initials: C
authors.lastname: Lucchesini
authors.forename: Francesca
authors.initials: F
authors.lastname: Ricci
authors.forename: Marzia
authors.initials: M
authors.lastname: Varettoni
authors.forename: Davide
authors.initials: D
authors.lastname: Filippini
authors.forename: Mario
authors.initials: M
authors.lastname: Lazzarino
authors.forename: Enrica
authors.initials: E
authors.lastname: Morra
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001799:Blood Sedimentation; D002471:Cell Transformation, Neoplastic; D003449:Cryoglobulinemia / Q000150:complications / Q000473:pathology* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis*; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000209:etiology / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications / Q000473:pathology* / Q000628:therapy; D012307:Risk Factors; D012737:Sex Factors; D016896:Treatment Outcome; D008258:Waldenstrom Macroglobulinemia / Q000209:etiology / Q000473:pathology
chemical_list: D007075:Immunoglobulin M