TY - JOUR
TI - International prognostic scoring system for Waldenstrom
macroglobulinemia
AU - Morel, Pierre
AU - Duhamel, Alain
AU - Gobbi, Paolo
AU - Dimopoulos,
AU - Meletios A.
AU - Dhodapkar, Madhav V.
AU - McCoy, Jason
AU - Crowley, John
AU - and Ocio, Enrique M.
AU - Garcia-Sanz, Ramon
AU - Treon, Steven P. and
AU - Leblond, Veronique
AU - Kyle, Robert A.
AU - Barlogie, Bart
AU - Merlini,
AU - Giampaolo
JO - Blood advances
PY - 2009
VL - 113
TODO - 18
SP - 4163-4170
PB - AMER SOC HEMATOLOGY
SN - null
TODO - 10.1182/blood-2008-08-174961
TODO - null
TODO - Recently, many new drugs have been developed for the treatment of
Waldenstrom 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, beta 2-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.
(Blood. 2009; 113: 4163-4170)
ER -