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DOI: 10.1055/a-1966-5765
Diagnostik und Therapie der alpha- und beta-Thalassämien
Diagnostics and Treatment of Alpha- and Beta-Thalassemias![](https://www.thieme-connect.de/media/transfusionsmedizin/202301/lookinside/thumbnails/19665765_10-1055-a-1966-5765-1.jpg)
Zusammenfassung
Die komplexe Behandlung von Patienten mit Thalassämien stellt nicht nur eine medizinische, sondern angesichts der in den letzten Jahren deutlich gestiegenen Patientenzahlen auch eine gesellschaftliche Herausforderung dar, die eine sehr enge Zusammenarbeit aller Behandler erfordert. Der vorliegende Beitrag erläutert Ursachen und Pathogenese der alpha- und beta-Thalassämien und bietet eine Übersicht zu Klinik und Therapien.
Abstract
Thalassemias are a heterogeneous group of genetic diseases based on a quantitative disorder of globin chain synthesis. They are among the most frequent monogenic hereditary diseases worldwide. Migration during recent years led to a profoundly increasing number of patients in countries where the indigenous population has not been affected. The complex treatment of the patients represents a medical and socioeconomic challenge with the need for structured interdisciplinary clinical care and close collaboration among healthcare providers, regulatory authorities, and health care insurance companies. The following article provides an overview of the causes, pathogenesis, clinical presentation, and treatment of alpha- and beta-thalassemias.
Schlüsselwörter
Hämoglobinopathie - α-Thalassämie - β-Thalassämie - Eisenüberladung - Hämochromatose - Chelat-TherapieKey words
hemoglobinopathy - α-thalassemia - β-thalassemia - iron overload - hemochromatosis - chelationPublikationsverlauf
Artikel online veröffentlicht:
21. Februar 2023
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