title: International prognostic scoring system for Waldenstrom macroglobulinemia.
abstract: 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 x 10(9)/L, beta2-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.
authors.affiliation: Hôpital Schaffner, Lens, France. pmorel@ch-lens.fr
authors.forename: Pierre
authors.initials: P
authors.lastname: Morel
authors.forename: Alain
authors.initials: A
authors.lastname: Duhamel
authors.forename: Paolo
authors.initials: P
authors.lastname: Gobbi
authors.forename: Meletios A
authors.initials: MA
authors.lastname: Dimopoulos
authors.forename: Madhav V
authors.initials: MV
authors.lastname: Dhodapkar
authors.forename: Jason
authors.initials: J
authors.lastname: McCoy
authors.forename: John
authors.initials: J
authors.lastname: Crowley
authors.forename: Enrique M
authors.initials: EM
authors.lastname: Ocio
authors.forename: Ramon
authors.initials: R
authors.lastname: Garcia-Sanz
authors.forename: Steven P
authors.initials: SP
authors.lastname: Treon
authors.forename: Veronique
authors.initials: V
authors.lastname: Leblond
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
authors.forename: Bart
authors.initials: B
authors.lastname: Barlogie
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
mesh_terms: D000367:Age Factors; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D038801:International Classification of Diseases; D008297:Male; D011379:Prognosis; D015996:Survival Rate; D016896:Treatment Outcome; D054928:Validation Studies as Topic; D008258:Waldenstrom Macroglobulinemia / Q000145:classification* / Q000175:diagnosis* / Q000188:drug therapy