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Immunoglobulin M Monoclonal Gammopathies of Undetermined Significance and Indolent Waldenström's Macroglobulinemia Recognize the Same Determinants of Evolution Into Symptomatic Lymphoid Disorders: Proposal for a Common Prognostic Scoring System

From the Unità Operativa Ematologia 1, Dipartimento di Ematologia e Oncologia, Ospedale Maggiore, I.R.C.C.S; Divisione di Medicina I, Ospedale "Fatebenefratelli e Oftalmico"; Divisione di Ematologia, Ospedale Niguarda, Cà Granda, Milan; Dipartimento di Medicina Interna e Oncologia Medica, Università degli Studi, Policlinico S. Matteo, I.R.C.C.S., Pavia; Divisione di Ematologia, Ospedali Riuniti, Bergamo; Dipartimento di Ematologia, Azienda Ospedaliera Bianchi-Melacrino-Morelli, Reggio Calabria; Dipartimento di Oncologia e Ematologia, Centro Oncologico Modenese, Università degli Studi, Policlinico, Modena; Divisione di Ematologia, Ospedale S. Gerardo, Monza; Servizio di Ematologia, Arcispedale S. Maria Nuova, Reggio Emila, Italy; Clinical Hematology Service of Hématologie Clinique, Hôpital Schaffner, Lens, France

Address reprint requests to Luca Baldini, MD, Unità Linfomi/Mielomi, Unità Operativa Ematologia 1, Centro G. Marcora, Ospedale Maggiore, I.R.C.C.S., Via F. Sforza 35, 20122 Milano, Italy; e-mail: lubaldini{at}policlinico.mi.it

PURPOSE: To evaluate the clinicohematologic variables at diagnosis that are prognostically related to neoplastic progression in patients with immunoglobulin M (IgM) monoclonal gammopathies of undetermined significance (MGUS), and indolent Waldenström's macroglobulinemia (IWM), and propose a scoring system to identify subsets of patients at different risk.

PATIENTS AND METHODS: We evaluated 217 patients with IgM MGUS and 201 with IWM (male-female ratio, 131:86 and 117:84; mean age, 63.7 and 63.6 years, respectively) diagnosed on the basis of serum monoclonal component (MC) levels and bone marrow lymphoplasmacytic infiltration degree. The variables selected by univariate analyses were multivariately investigated; on the basis of their individual relative hazards, a scoring system was devised to identify subsets of patients at different risk of evolution.

RESULTS: After a median follow-up of 56.1 and 60.2 months, 15 of 217 MGUS and 45 of 201 IWM patients, respectively, required chemotherapy for symptomatic WM (13 and 36), non-Hodgkin's lymphoma (2 and 6) and amyloidosis (0 and 3). The median time to evolution (TTE) was not reached for MGUS and was 141.5 months for IWM. The variables adversely related to evolution were qualitatively the same in both groups: MC levels, Hb concentrations and sex. A scoring system based on these parameters identified three risk groups with highly significant differences in TTE in both groups (P < .0001).

CONCLUSION: MGUS and IWM identify disease entities with different propensities for symptomatic neoplastic evolution. As both have the same prognostic determinants of progression, we propose a practical scoring system that, identifying different risks of malignant evolution, may allow an individualized clinical approach.

Supported by a grant (Ricerca Corrente, Progetti a Concorso) from the Italian Ministry of Health to Ospedale Maggiore IRCCS, Milan, Italy.

A complete list of the Gruppo Italiano Studio Linfomi (GISL) Centres who referred patients to the study is given at the end of this article.

Authors' disclosures of potential conflicts of interest are found at the end of this article.

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