VEXAS : is it time to reshape the nosology of clonal hematopoiesis?
INTRODUCTION: The recent description of VEXAS (for vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) challenges the nosological framework of myelodysplastic syndromes.
AREAS COVERED: Clonal expansion driven by somatic mutations in cancer genes has been largely described in healthy aging individuals. Regarding hematopoiesis, the prevalence of clonal hematopoiesis has blurred the line between normal and pathological, especially for the definition of myelodysplastic syndromes. VEXAS syndrome further challenges the nosology as this clonal disease of hematopoiesis is also associated with dysplastic features and cytopenias.
EXPERT OPINION: In this perspective, we discuss whether VEXAS should be considered a genuine myelodysplastic syndrome and propose a conceptual framework to refine the nosology, based on the distinction of clonal hematopoiesis of indeterminate potential (CHIP), clonal hematopoiesis of hematological significance, and clonal hematopoiesis of other significance.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:16 |
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| Enthalten in: |
Expert review of hematology - 16(2023), 7 vom: 29. Juli, Seite 495-499 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Sujobert, Pierre [VerfasserIn] |
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| Links: |
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| Themen: |
Clonal hematopoiesis |
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| Anmerkungen: |
Date Completed 23.06.2023 Date Revised 25.06.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1080/17474086.2023.2209715 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| 520 | |a INTRODUCTION: The recent description of VEXAS (for vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) challenges the nosological framework of myelodysplastic syndromes | ||
| 520 | |a AREAS COVERED: Clonal expansion driven by somatic mutations in cancer genes has been largely described in healthy aging individuals. Regarding hematopoiesis, the prevalence of clonal hematopoiesis has blurred the line between normal and pathological, especially for the definition of myelodysplastic syndromes. VEXAS syndrome further challenges the nosology as this clonal disease of hematopoiesis is also associated with dysplastic features and cytopenias | ||
| 520 | |a EXPERT OPINION: In this perspective, we discuss whether VEXAS should be considered a genuine myelodysplastic syndrome and propose a conceptual framework to refine the nosology, based on the distinction of clonal hematopoiesis of indeterminate potential (CHIP), clonal hematopoiesis of hematological significance, and clonal hematopoiesis of other significance | ||
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| 700 | 1 | |a Kosmider, Olivier |e verfasserin |4 aut | |
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