A prognostic index predicting survival in transformed Waldenström macroglobulinemia

Eric Durot, Lukshe Kanagaratnam, Saurabh Zanwar, Efstathios Kastritis, Shirley D’Sa, Ramon Garcia-Sanz, Cécile Tomowiak, Bénédicte Hivert, Elise Toussaint, Caroline Protin, Jithma P. Abeykoon, Thomas Guerrero-Garcia, Gilad Itchaki, Josephine M. Vos, Anne Sophie Michallet, Sophie Godet, Jehan Dupuis, Stéphane Leprêtre, Joshua Bomsztyk, Pierre MorelVéronique Leblond, Steven P. Treon, Meletios A. Dimopoulos, Prashant Kapoor, Alain Delmer, Jorge J. Castillo

Research output: Contribution to journalArticlepeer-review


istological transformation into diffuse large B-cell lymphoma is a rare complication in patients with Waldenström macroglobulinemia (WM) and is usually associated with a poor prognosis. The objective of this study was to develop and validate a prognostic index for survival of patients with transformed WM. Through this multicenter, international collaborative effort, we developed a scoring system based on data from 133 patients with transformed WM who were evaluated between 1995 and 2016 (training cohort). Univariate and multivariate analyses were used to propose a prognostic index with 2-year survival after transformation as an endpoint. For external validation, a dataset of 67 patients was used to evaluate the performance of the model (validation cohort). By multivariate analysis, three adverse covariates were identified as independent predictors of 2-year survival after transformation: elevated serum lactate dehydrogenase (2 points), platelet count <100x109/L (1 point) and any previous treatment for WM (1 point). Three risk groups were defined: low-risk (0-1 point, 24% of patients), intermediate-risk (2-3 points, 59%; hazard ratio = 3.4) and high-risk (4 points, 17%; hazard ratio = 7.5). Two-year survival rates were 81%, 47%, and 21%, respectively (P<0.0001). This model appeared to be a better discriminant than either the International Prognostic Index or the revised International Prognostic Index. We validated this model in an independent cohort. This easy-to-compute scoring index is a robust tool that may allow identification of groups of transformed WM patients with different outcomes and could be used for improving the development of risk-adapted treatment strategies.

Original languageEnglish (US)
Pages (from-to)2940-2946
Number of pages7
Issue number11
StatePublished - Nov 2021

ASJC Scopus subject areas

  • Hematology


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