A revised international prognostic score system for Waldenström’s macroglobulinemia

Kastritis, Efstathios; Morel, Pierre; Duhamel, Alain; Gavriatopoulou, Maria; Kyrtsonis, Marie Christine; Durot, Éric; Symeonidis, Argiris; Laribi, Kamel; Hatjiharissi, Evdoxia; Ysebaert, Loïc; Vassou, Amalia; Giannakoulas, Nikolaos; Merlini, Giampaolo; Repousis, Panagiotis; Varettoni, Marzia; Michalis, E.; Hivert, Bénédicte; Michail, Michalis; Katodritou, Eirini; Terpos, Evangelos; Leblond, Véronique

doi:10.1038/s41375-019-0431-y

Cited by 73 publications

(55 citation statements)

References 26 publications

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“…[14][15][16][17] Its prognostic role has been validated in both WM and DLBCL, being one of the variables included in the revised IPSSWM and the IPI, respectively. 6,18 Low platelet count, also part of the IPSSWM, is usually associated with a poor prognosis in hematological malignancies 12,19 and could reflect a critical level of bone marrow involvement. For development of the prognostic score, only pretreatment characteristics were considered.…”

Section: Discussionmentioning

confidence: 99%

A prognostic index predicting survival in transformed Waldenström macroglobulinemia

et al. 2020

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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.

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Section: Discussionmentioning

confidence: 99%

A prognostic index predicting survival in transformed Waldenström macroglobulinemia

et al. 2020

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“…This finding strengthens the clinical significance of the genetic background of the CXCL12/CXCR4 axis in LPL/WM, and prospective studies incorporating CXCL12 polymorphisms and CXCR4 mutation status in assessing treatment response, progression-free survival, and overall survival in LPL/WM patients could nonetheless clarify the predictive and prognostic roles of CXCL12 and CXCR4 variants, both alone and together. Unfortunately, to date the revised International Prognostic Scoring System for Waldenström Macroglobulinemia (IPSSWM) does not include the mutational status of either MYD88 or CXCR4 [156], as it is not routinely performed and is available in only a few patients. It has been associated with prognosis in some studies [85] but not confirmed in others [123], and it has been considered as predictive for response to ibrutinib [157,158].…”

Section: Cxcr4 Whim-like Mutations In Lpl/wmmentioning

confidence: 99%

New Insights on the Emerging Genomic Landscape of CXCR4 in Cancer: A Lesson from WHIM

et al. 2020

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Deciphering the molecular alterations leading to disease initiation and progression is currently crucial to identify the most relevant targets for precision therapy in cancer patients. Cancers express a complex chemokine network influencing leucocyte infiltration and angiogenesis. Moreover, malignant cells also express a selective repertoire of chemokine receptors that sustain their growth and spread. At present, different cancer types have been shown to overexpress C-X-C chemokine receptor type 4 (CXCR4) and to respond to its ligand C-X-C motif chemokine 12 (CXCL12). The CXCL12/CXCR4 axis influences cancer biology, promoting survival, proliferation, and angiogenesis, and plays a pivotal role in directing migration of cancer cells to sites of metastases, making it a prognostic marker and a therapeutic target. More recently, mutations in the C-terminus of CXCR4 have been identified in the genomic landscape of patients affected by Waldenstrom’s macroglobulinemia, a rare B cell neoplasm. These mutations closely resemble those occurring in Warts, Hypogammaglobulinemia, Immunodeficiency, and Myelokathexis (WHIM) syndrome, an immunodeficiency associated with CXCR4 aberrant expression and activity and with chemotherapy resistance in clinical trials. In this review, we summarize the current knowledge on the relevance of CXCR4 mutations in cancer biology, focusing on its importance as predictors of clinical presentation and response to therapy.

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“…A prognostic system International Prognostic Scoring System for WM (IPSSWM), based on age, b2-microglobulin, hemoglobin, platelet counts, and IgM level, stratifies patients into 3 risk groups. 35 Recently, a revised score (rIPSSWM) was developed 36 on the basis of age, b2-microglobulin, serum LDH, and albumin, and identifies 5 prognostic groups. In IPSSWM and rIPSSWM, age is a critical prognostic determinant, and both are based on biochemical parameters and not molecular or genetic characteristics.…”

Section: Risk Assessment In Symptomatic Wmmentioning

confidence: 99%

How I treat Waldenström macroglobulinemia

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Kastritis²

2019

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A revised international prognostic score system for Waldenström’s macroglobulinemia

Cited by 73 publications

References 26 publications

A prognostic index predicting survival in transformed Waldenström macroglobulinemia

A prognostic index predicting survival in transformed Waldenström macroglobulinemia

New Insights on the Emerging Genomic Landscape of CXCR4 in Cancer: A Lesson from WHIM

How I treat Waldenström macroglobulinemia

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