MARS: mutation-adjusted risk score for advanced systemic mastocytosis
PURPOSE: To develop a risk score for patients with advanced systemic mastocytosis (AdvSM) that integrates clinical and mutation characteristics. PATIENTS AND METHODS: The study included 383 patients with AdvSM from the German Registry on Disorders of Eosinophils and Mast Cells (training set; n = 231...
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| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
September 11, 2019
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| In: |
Journal of clinical oncology
Year: 2019, Volume: 37, Issue: 31, Pages: 2846-2856 |
| ISSN: | 1527-7755 |
| DOI: | 10.1200/JCO.19.00640 |
| Online Access: | Verlag, Volltext: https://doi.org/10.1200/JCO.19.00640 Verlag: https://ascopubs.org/doi/full/10.1200/JCO.19.00640 
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| Author Notes: | Mohamad Jawhar, Juliana Schwaab, Iván Álvarez-Twose, Khalid Shoumariyeh, Nicole Naumann, Johannes Lübke, Cecelia Perkins, Javier I. Muñoz-González, Manja Meggendorfer, Vanessa Kennedy, Georgia Metzgeroth, Alice Fabarius, Dietmar Pfeifer, Karl Sotlar, Hans-Peter Horny, Nikolas von Bubnoff, Torsten Haferlach, Nicholas C.P. Cross, Wolf-Karsten Hofmann, Wolfgang R. Sperr, Andrés C. García-Montero, Peter Valent, Jason Gotlib, Alberto Orfao, Andreas Reiter |
MARC
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| 245 | 1 | 0 | |a MARS |b mutation-adjusted risk score for advanced systemic mastocytosis |c Mohamad Jawhar, Juliana Schwaab, Iván Álvarez-Twose, Khalid Shoumariyeh, Nicole Naumann, Johannes Lübke, Cecelia Perkins, Javier I. Muñoz-González, Manja Meggendorfer, Vanessa Kennedy, Georgia Metzgeroth, Alice Fabarius, Dietmar Pfeifer, Karl Sotlar, Hans-Peter Horny, Nikolas von Bubnoff, Torsten Haferlach, Nicholas C.P. Cross, Wolf-Karsten Hofmann, Wolfgang R. Sperr, Andrés C. García-Montero, Peter Valent, Jason Gotlib, Alberto Orfao, Andreas Reiter |
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| 520 | |a PURPOSE: To develop a risk score for patients with advanced systemic mastocytosis (AdvSM) that integrates clinical and mutation characteristics. PATIENTS AND METHODS: The study included 383 patients with AdvSM from the German Registry on Disorders of Eosinophils and Mast Cells (training set; n = 231) and several centers for mastocytosis in the United States and Europe, all within the European Competence Network on Mastocytosis (validation set; n = 152). A Cox multivariable model was used to select variables that were predictive of overall survival (OS). RESULTS: In multivariable analysis, the following risk factors were identified as being associated with OS: age greater than 60 years, anemia (hemoglobin < 10 g/dL), thrombocytopenia (platelets < 100 × 109/L), presence of one high molecular risk gene mutation (ie, in SRSF2, ASXL1, and/or RUNX1), and presence of two or more high molecular risk gene mutations. By assigning hazard ratio-weighted points to these variables, the following three risk categories were defined: low risk (median OS, not reached), intermediate risk (median OS, 3.9 years; 95% CI, 2.1 to 5.7 years), and high risk (median OS, 1.9 years; 95% CI, 1.3 to 2.6 years; P < .001). The mutation-adjusted risk score (MARS) was independent of the WHO classification and was confirmed in the independent validation set. During a median follow-up time of 2.2 years (range, 0 to 23 years), 63 (16%) of 383 patients experienced a leukemic transformation to secondary mast cell leukemia (32%) or secondary acute myeloid leukemia (68%). The MARS was also predictive for leukemia-free survival (P < .001).CONCLUSIONThe MARS is a validated, five-parameter, WHO-independent prognostic score that defines three risk groups among patients with AdvSM and may improve up-front treatment stratification for these rare hematologic neoplasms. | ||
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