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A prognostic index predicting survival in transformed Waldenström macroglobulinemia

Durot, E; Kanagaratnam, L; Zanwar, S; Kastritis, E; D'Sa, S; Garcia-Sanz, R; Tomowiak, C; ... Castillo, JJ; + view all (2020) A prognostic index predicting survival in transformed Waldenström macroglobulinemia. Haematologica , 106 (11) 10.3324/haematol.2020.262899. Green open access

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Abstract

Histological transformation into diffuse large B-cell lymphoma is a rare complication in patients with Waldenström macroglobulinemia (WM) usually associated with a poor prognosis. The objective of this study was to develop and validate a prognostic index for survival in transformed WM patients. 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 end-point. For external validation, a data set 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 LDH (2 points), platelet count < 100 x 109/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 (HR) = 3.4) and high-risk (4 points, 17%, HR = 7.5). Two-year survival rates were 81%, 47%, and 21%, respectively (P < 0.0001). This model appeared to be a better discriminant than the International Prognostic Index (IPI) and the revised IPI (R-IPI). 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.

Type: Article
Title: A prognostic index predicting survival in transformed Waldenström macroglobulinemia
Location: Italy
Open access status: An open access version is available from UCL Discovery
DOI: 10.3324/haematol.2020.262899
Publisher version: https://doi.org/10.3324/haematol.2020.262899
Language: English
Additional information: This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Keywords: Humans, Lymphoma, Large B-Cell, Diffuse, Prognosis, Proportional Hazards Models, Survival Rate, Waldenstrom Macroglobulinemia
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10132696
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