@article{3077581,
    title = "A revised international prognostic score system for Waldenström’s macroglobulinemia",
    author = "Kastritis, E. and Morel, P. and Duhamel, A. and Gavriatopoulou, M. and Kyrtsonis, M.C. and Durot, E. and Symeonidis, A. and Laribi, K. and Hatjiharissi, E. and Ysebaert, L. and Vassou, A. and Giannakoulas, N. and Merlini, G. and Repousis, P. and Varettoni, M. and Michalis, E. and Hivert, B. and Michail, M. and Katodritou, E. and Terpos, E. and Leblond, V. and Dimopoulos, M.A.",
    journal = "Leukemia Research",
    year = "2019",
    volume = "33",
    number = "11",
    pages = "2654-2661",
    publisher = "Nature Publishing Group",
    issn = "0145-2126",
    doi = "10.1038/s41375-019-0431-y",
    keywords = "albumin;  beta 2 microglobulin;  immunoglobulin M;  lactate dehydrogenase;  rituximab;  rituximab, adolescent;  adult;  aged;  albumin blood level;  Article;  cancer prognosis;  cancer staging;  child;  cohort analysis;  controlled study;  disease exacerbation;  high risk patient;  high risk population;  human;  major clinical study;  mortality rate;  multivariate analysis;  overall survival;  patient risk;  peripheral neuropathy;  priority journal;  scoring system;  survival rate;  symptomatology;  validation process;  very elderly;  Waldenstroem macroglobulinemia;  follow up;  hematology;  immunotherapy;  international cooperation;  middle aged;  oncology;  procedures;  prognosis;  proportional hazards model;  prospective study;  recurrent disease;  risk assessment;  severity of illness index;  Waldenstroem macroglobulinemia, Adult;  Aged;  Aged, 80 and over;  Follow-Up Studies;  Hematology;  Humans;  Immunotherapy;  International Cooperation;  Medical Oncology;  Middle Aged;  Neoplasm Staging;  Prognosis;  Proportional Hazards Models;  Prospective Studies;  Recurrence;  Risk Assessment;  Rituximab;  Severity of Illness Index;  Survival Rate;  Waldenstrom Macroglobulinemia",
    abstract = "A staging system was developed a decade ago for patients with Waldenström’s macroglobulinemia (WM), however, since then WM treatments have changed. A revised staging system could better capture prognosis of WM patients in the chemoimmunotherapy era. We developed a revised system based on data from 492 symptomatic patients with at least 3 years and a median of 7 years of follow up while an independent validation cohort included 229 symptomatic patients. We identified age (≤65 vs 66–75 vs ≥76 years), b2-microglobulin ≥ 4 mg/L, serum albumin <3.5 gr/dl, and LDH ≥ 250 IU/L (ULN < 225) to stratify patients in five different prognostic groups and identify a very-low risk as well as a very-high risk group with a 3-year WM-related death rate of 0, 10, 14, 38, and 48% (p < 0.001) and 10-year survival rate of 84, 59, 37, 19, and 9% (p < 0.001). We evaluated this staging system separately in patients >65 years and <65 years, according to the reason for initiation of treatment, among patients receiving frontline rituximab or in patients treated primarily without rituximab. With further validation before clinical use, this revised IPSSWM could improve WM patient risk stratification, is easily available and may be used in the everyday practice to provide prognostic information. © 2019, Springer Nature Limited."
}