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Hamostaseologie 1994; 14(02): 60-68
DOI: 10.1055/s-0038-1660345
Übersichtsarbeit/Review Articles
Schattauer GmbH

Moderne Therapieformen zur Behandlung von Hämophilie

Vom Dunkel ins Licht
P. M. Mannucci
1   Angelo Bianchi Bonomi Hämophilie und Thrombose Zentrum und Institut für Innere Medizin, Universität von Milano, Milano, Italien
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Publication History

Publication Date:
26 June 2018 (online)

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Zusammenfassung

In den vergangenen Jahren konnten bedeutende Fortschritte im Bereich der Behandlung von Hämophilie verzeichnet werden, die zweifelsohne als eine Reaktion der Wissenschaft auf den Alptraum der AIDS-Epidemie gesehen werden können. Gerinnungsfaktorkonzentrate werden immer sicherer, und somit wird gleichzeitig das Übertragungsrisiko von hämatogenen Infektionen immer geringer. Das Risiko einer HIV-lnfektion kann, wenn man menschliches Versagen ausschließt, als unbedeutend betrachtet werden. Ein geringes Restrisiko einer Übertragung von Hepatitisviren besteht noch, diesem sollte jedoch mit verbesserten Methoden zur Virusinaktivierung begegnet werden. Auch die Reinheit plasmatischer Konzentrate wird ständig erhöht. Einige Untersuchungsergebnisse deuten darauf hin, daß ultrareine, monoklonal gereinigte Plasmaprodukte bei HlV-seropositiven Blutern zu einer Stabilisierung der CD4-Lymphozytenzahl führen. Faktor VIII, der mittels rekombinanter Gentechnologie hergestellt wurde, ist in einigen Ländern bereits lizensiert. Auf diesem Weg gewonnener Faktor VIII ist von hoher Wirksamkeit und -so bleibt zu hoffen - frei von jeglichem Risiko einer Virenübertragung. Über die Frage, ob diese Produkte das Risiko einer Hemmkörperbildung erhöhen, wird immer noch diskutiert. Abschließend wäre zu bemerken, daß die gegenwärtig explosionsartig fortschreitende Entwicklung der Molekularbiologie auf eine erfolgreiche Entwicklung einer Gentherapie zur Behandlung von Hämophilie noch in diesem Jahrzehnt hoffen läßt.

Schlüsselwörter

Hämophilie - Gerinnungsfaktorkonzentrate - Virussicherheit - rekombinanter Faktor VIII
 

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