International prognostic scoring system for Waldenström 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, Giampaolo Merlini

Research output: Contribution to journalArticlepeer-review


Recently, many new drugs have been developed for the treatment of Waldenström macroglobulinemia (WM). To optimize the treatment according to the prognosis and to facilitate the comparison of trials, we developed an International Prognostic Scoring System for WM in a series of 587 patients with clearly defined criteria for diagnosis and for initiation of treatment. The median survival after treatment initiation was 87 months. Five adverse covariates were identified: advanced age (>65 years), hemoglobin less than or equal to 11.5 g/dL, platelet count less than or equal to 100 × 10 9/L, β2-microglobulin more than 3 mg/L, and serum monoclonal protein concentration more than 7.0 g/dL. Low-risk patients (27%) presented with no or 1 of the adverse characteristics and advanced age, intermediate-risk patients (38%) with 2 adverse characteristics or only advanced age, and high-risk patients (35%) with more than 2 adverse characteristics. Five-year survival rates were 87%, 68%, and 36%, respectively (P <.001). The ISSWM retained its prognostic significance in subgroups defined by age, treatment with alkylating agent, and purine analog. Thus, the ISSWM may provide a means to design risk-adapted studies. However, independent validation and new biologic markers may enhance its significance.

Original languageEnglish
Pages (from-to)4163-4170
Number of pages8
Issue number18
Publication statusPublished - 2009

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology


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