| |  Online-Ressource |
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| Verfasst von: | Jawhar, Mohamad [VerfasserIn]  |
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| | Schwaab, Juliana [VerfasserIn] |
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| | Álvarez-Twose, Iván [VerfasserIn] |
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| | Shoumariyeh, Khalid [VerfasserIn] |
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| | Naumann, Nicole [VerfasserIn] |
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| | Lübke, Johannes [VerfasserIn] |
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| | Perkins, Cecelia [VerfasserIn] |
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| | Muñoz-Gonźalez, Javier Ignacio [VerfasserIn] |
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| | Meggendorfer, Manja [VerfasserIn] |
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| | Kennedy, Vanessa [VerfasserIn] |
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| | Metzgeroth, Georgia [VerfasserIn] |
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| | Fabarius, Alice [VerfasserIn] |
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| | Pfeifer, Dietmar [VerfasserIn] |
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| | Sotlar, Karl [VerfasserIn] |
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| | Horny, Hans-Peter [VerfasserIn] |
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| | von Bubnoff, Nikolas [VerfasserIn] |
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| | Haferlach, Torsten [VerfasserIn] |
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| | Cross, Nicholas C.P. [VerfasserIn] |
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| | Hofmann, Wolf-Karsten [VerfasserIn] |
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| | Sperr, Wolfgang R. [VerfasserIn] |
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| | García-Montero, Andrés C. [VerfasserIn] |
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| | Valent, Peter [VerfasserIn] |
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| | Gotlib, Jason [VerfasserIn] |
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| | Orfao, Alberto [VerfasserIn] |
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| | Reiter, Andreas [VerfasserIn] |
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| Titel: | MARS |
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| Titelzusatz: | mutation-adjusted risk score for advanced systemic mastocytosis |
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| Verf.angabe: | 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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| E-Jahr: | 2019 |
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| Jahr: | September 11, 2019 |
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| Umfang: | 11 S. |
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| Teil: | volume:37 |
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| | year:2019 |
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| | number:31 |
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| | pages:2846-2856 |
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| | extent:11 |
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| Fussnoten: | Gesehen am 02.03.2020 |
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| Titel Quelle: | Enthalten in: Journal of clinical oncology |
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| Ort Quelle: | Alexandria, Va. : American Society of Clinical Oncology, 1983 |
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| Jahr Quelle: | 2019 |
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| Band/Heft Quelle: | 37(2019), 31, Seite 2846-2856 |
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| ISSN Quelle: | 1527-7755 |
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| Abstract: | 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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| DOI: | doi:10.1200/JCO.19.00640 |
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| URL: | Bitte beachten Sie: Dies ist ein Bibliographieeintrag. Ein Volltextzugriff für Mitglieder der Universität besteht hier nur, falls für die entsprechende Zeitschrift/den entsprechenden Sammelband ein Abonnement besteht oder es sich um einen OpenAccess-Titel handelt.
Volltext: https://doi.org/10.1200/JCO.19.00640 |
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| | Verlag: https://ascopubs.org/doi/full/10.1200/JCO.19.00640 |
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| | DOI: https://doi.org/10.1200/JCO.19.00640 |
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| Datenträger: | Online-Ressource |
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| Sprache: | eng |
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| K10plus-PPN: | 1691302449 |
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| Verknüpfungen: | → Zeitschrift |
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