title: Risk of malignant transformation in patients with monoclonal gammopathy of undetermined significance.
abstract: The acturial probability of malignant transformation was analyzed in a series of 263 patients with monoclonal gammopathy of undetermined significance (MGUS) over a 15-year period and followed from 5 to 20 years. At a median follow-up of 11.5 years, 157 patients (59.7%) had died of causes unrelated to MGUS, 47 (17.9%) were still alive and presented no increase in monoclonal component, 11 (4.1%) presented an increase in monoclonal component without evidence of malignant immunoproliferative disease, and 48 (18.3%) had developed a malignant transformation of MGUS. In particular, MGUS evolved into 35 cases of multiple myeloma, two of solitary plasmacytoma of the bone, four of macroglobulinemia, three of malignant lymphoma, two of amyloidosis, one of chronic lymphocytic leukemia, and one of plasma cell leukemia. The cumulative incidence of malignant transformation was 18.3%; and the actuarial risk of malignant transformation was 6.1, 15.4, and 31.3% at 5, 10 and 20 years, respectively. The multivariate regression analysis according to Cox's proportional hazard model selected among 22 different variables established at initial diagnosis of MGUS only age as the factor significantly (P < 0.011) and negatively (b = -1.104) related to the risk of developing a malignant immunoproliferative disease. Therefore, patients with MGUS present an increased risk of developing a malignant lymphoproliferative or plasma cell proliferative disease, and MGUS could be considered a pre-neoplastic condition. Since no clinical or laboratory features are able to identify in advance the patients at high risk of disease progression, each patient must be followed up periodically and over an indefinite period.
authors.affiliation: Department of Internal Medicine and Public Health, School of Medicine and Surgery, University of L'Aquila, Italy.
authors.forename: P
authors.initials: P
authors.lastname: Pasqualetti
authors.forename: R
authors.initials: R
authors.lastname: Casale
mesh_terms: D000206:Actuarial Analysis; D000368:Aged; D002471:Cell Transformation, Neoplastic; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007160:Immunoproliferative Disorders / Q000453:epidemiology* / Q000209:etiology; D008875:Middle Aged; D015999:Multivariate Analysis; D010265:Paraproteinemias / Q000150:complications / Q000473:pathology*; D012307:Risk Factors