| |  Online-Ressource |
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| Verfasst von: | Naumann, Nicole [VerfasserIn]  |
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| | Rudelius, Martina [VerfasserIn] |
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| | Lübke, Johannes [VerfasserIn] |
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| | Christen, Deborah [VerfasserIn] |
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| | Bresser, Jakob [VerfasserIn] |
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| | Sotlar, Karl [VerfasserIn] |
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| | Metzgeroth, Georgia [VerfasserIn] |
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| | Fabarius, Alice [VerfasserIn] |
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| | Hofmann, Wolf-Karsten [VerfasserIn] |
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| | Panse, Jens [VerfasserIn] |
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| | Horny, Hans-Peter [VerfasserIn] |
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| | Cross, Nicholas C. P. [VerfasserIn] |
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| | Reiter, Andreas [VerfasserIn] |
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| | Schwaab, Juliana [VerfasserIn] |
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| Titel: | Poor applicability of currently available prognostic scoring systems for prediction of outcome in KIT D816V-negative advanced systemic mastocytosis |
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| Verf.angabe: | Nicole Naumann, Martina Rudelius, Johannes Lübke, Deborah Christen, Jakob Bresser, Karl Sotlar, Georgia Metzgeroth, Alice Fabarius, Wolf-Karsten Hofmann, Jens Panse, Hans-Peter Horny, Nicholas C.P. Cross, Andreas Reiter and Juliana Schwaab |
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| E-Jahr: | 2024 |
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| Jahr: | 30 January 2024 |
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| Umfang: | 18 S. |
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| Illustrationen: | Illustrationen |
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| Fussnoten: | Gesehen am 18.06.2024 |
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| Titel Quelle: | Enthalten in: Cancers |
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| Ort Quelle: | Basel : MDPI, 2009 |
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| Jahr Quelle: | 2024 |
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| Band/Heft Quelle: | 16(2024), 3, Artikel-ID 593, Seite 1-18 |
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| ISSN Quelle: | 2072-6694 |
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| Abstract: | Within our nationwide registry, we identified a KIT D816V mutation (KIT D816Vpos.) in 280/299 (94%) patients with advanced systemic mastocytosis (AdvSM). Age, cytopenias and the presence of additional somatic mutations confer inferior overall survival (OS). However, little is known about the characteristics of KIT D816V-negative (D816Vneg.) AdvSM. In 19 D816Vneg. patients, a combination of clinical, morphological and genetic features revealed three subgroups: (a) KIT D816H- or Y-positive SM (KIT D816H/Ypos., n = 7), predominantly presenting as mast cell leukemia (MCL; 6/7 patients), (b) MCL with negative KIT sequencing (KITneg. MCL, n = 7) and (c) KITneg. SM with associated hematologic neoplasm (KITneg. SM-AHN, n = 5). Although >70% of patients in the two MCL cohorts (KIT D816H/Ypos. and KITneg.) were classified as low/intermediate risk according to prognostic scoring systems (PSS), treatment response was poor and median OS was shorter than in a KIT D816Vpos. MCL control cohort (n = 29; 1.7 vs. 0.9 vs. 2.6 years; p < 0.04). The KITneg. SM-AHN phenotype was dominated by the heterogeneous AHN (low mast cell burden, presence of additional mutations) with a better median OS of 4.5 years. We conclude that (i) in MCL, negativity for D816V is a relevant prognostic factor and (ii) PSS fail to correctly classify D816Vneg. patients. |
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| DOI: | doi:10.3390/cancers16030593 |
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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.
kostenfrei: Volltext: https://doi.org/10.3390/cancers16030593 |
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| | kostenfrei: Volltext: https://www.mdpi.com/2072-6694/16/3/593 |
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| | DOI: https://doi.org/10.3390/cancers16030593 |
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| Datenträger: | Online-Ressource |
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| Sprache: | eng |
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| Sach-SW: | KIT D816H |
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| | KIT D816V-negative |
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| | KIT D816Y |
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| | KIT mutation-negative |
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| | advanced systemic mastocytosis |
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| K10plus-PPN: | 1891431854 |
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| Verknüpfungen: | → Zeitschrift |
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Poor applicability of currently available prognostic scoring systems for prediction of outcome in KIT D816V-negative advanced systemic mastocytosis / Naumann, Nicole [VerfasserIn]; 30 January 2024 (Online-Ressource)
