@article{3032521,
    title = "A prognostic index predicting survival in transformed Waldenstrom
macroglobulinemia",
    author = "Durot, Eric and Kanagaratnam, Lukshe and Zanwar, Saurabh and Kastritis, and Efstathios and D'Sa, Shirley and Garcia-Sanz, Ramon and Tomowiak, Cecile and and Hivert, Benedicte and Toussaint, Elise and Protin, Caroline and and Abeykoon, Jithma P. and Guerrero-Garcia, Thomas and Itchaki, Gilad and and Vos, Josephine M. and Michallet, Anne-Sophie and Godet, Sophie and and Dupuis, Jehan and Lepretre, Stephane and Bomsztyk, Joshua and Morel, and Pierre and Leblond, Veronique and Treon, Steven P. and Dimopoulos, and Meletios A. and Kapoor, Prashant and Delmer, Alain and Castillo, Jorge and J.",
    journal = "Haematologica-the hematology journal",
    year = "2021",
    volume = "106",
    number = "11",
    pages = "2940-2946",
    publisher = "Ferrata Storti Foundation",
    doi = "10.3324/haematol.2020.262899",
    abstract = "Histological transformation into diffuse large B-cell lymphoma is a rare
complication in patients with Waldenstrom 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 <100x10(9)/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."
}