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Blood, 30 April 2009, Vol. 113, No. 18, pp. 4163-4170.
Prepublished online as a Blood First Edition Paper on February 5, 2009; DOI 10.1182/blood-2008-08-174961.


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Submitted August 21, 2008
Accepted December 27, 2008

International prognostic scoring system for Waldenstrom's macroglobulinemia

Pierre Morel*, Alain Duhamel, Paolo Gobbi, Meletios A. Dimopoulos, Madhav V. Dhodapkar, Jason McCoy, John Crowley, Enrique M. Ocio, Ramon Garcia-Sanz, Steven P. Treon, Veronique Leblond, Robert A. Kyle, Bart Barlogie, and Giampaolo Merlini

Hopital Schaffner, Lens, France
Faculte de Medecine, Lille, France
Fondazione IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy
University of Athens School of Medicine, Athens, Greece
The Rockefeller University, New York, NY, United States
Southwest Oncology Group, Seattle, WA, United States
Spanish Group for the Study of Waldenstrom Macroglobulinaemia, Salamanca, Spain
Dana Farber Cancer Institute, Boston, MA, United States
French cooperative group Chronic Lymphocytic leukaemia/Waldenstrom Macroglobulinemia, Paris, France
Mayo Clinic, Rochester, MN, United States
University of Arkansas for Medical Sciences, Little Rock, AR, United States

* Corresponding author; email: pmorel{at}ch-lens.fr.

Recently, many new drugs have been developed for the treatment of Waldenstrom's macroglobulinemia (WM). In order to optimize the treatment according to the prognosis and to facilitate the comparison of trials we developed an international scoring system for WM (ISSWM) in a series of 587 patients with clearly defined criteria for diagnosis and for initiation of treatment. With a median follow-up of 64 months, the median survival after treatment initiation was 87 months. Five adverse covariates were identified: age >65 years, hemoglobin ≤11·5 g/dL, platelet count ≤100 x 109/L, {beta}2-microglobulin >3 mg/L and serum monoclonal protein concentration >7.0 g/dL. Low risk patients (27% of patients) presented with ≤1 adverse characteristic and age ≤65 years, intermediate risk patients (38%) with 2 adverse characteristics or only age >65 years and high risk patients (35%) with >2 adverse characteristics. Five-year survival rates were 87%, 68% and 36% respectively (p<0·0001). The ISSWM retained its prognostic significance in subgroups defined by age ≤65 years, >65 years, treatment with alkylating agent, and purine analogue. Thus, the combination of age, {beta}2-microglobulin, monoclonal protein concentration and blood counts may provide a means to design risk-adapted studies. However, independent validation and new biological markers may enhance its significance.


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