Zusammenfassung
An einem Fall von paroxysmaler nächtlicher Hämoglobinurie konnte der Einfluß des Thrombins sowie der hämolysehemmenden Faktoren des inaktivierten Serums auf die Hämolyse geprüft werden. Während der hitzestabile SerumfaktorCrosbys über das hämolytische Serumsystem zu einer Hemmung der Hämolyse von PNH-Erythrozyten führt, setzt die Wirkung des die Hämolyse hemmenden Faktors vonKirchmayer undSzczepkowski («KS-Faktor») an den PNH-Erythrozyten selbst an. Thrombin bewirkt eine Steigerung der Hämolysetendenz der Erythrozyten, und zwar infolge eines direkten Angriffs auf die Erythrozyten. Es handelt sich vermutlich um einen fermentativen Vorgang an der Zelloberfläche, wie ihn in analoger Weise auchYachnin, Laforet undGardner an Normalerythrozyten beobachten konnten. Eine Beeinflussung der hämolysehemmenden Serumfaktoren durch Thrombin, wie sie im Gegensatz zuKirchmayer undSzczepkowski vonCrosby angenommen wird, konnte nicht festgestellt werden. Die Bedeutung von Faktoren des Gerinnungssystems für die Hämolyse von PNH-Erythrozyten in vivo unterstreicht der therapeutische Erfolg einer Dauerbehandlung mit Dicumarol.
Summary
The effects of thrombin and of factors inhibiting haemolysis in inactivated serum have been investigated in a case of paroxysmal nocturnal haemoglobinuria. The heat-stable serum factor ofCrosby inhibits haemolysis of PNH corpuscles by an effect on the haemolytic system of the serum, whereas the inhibitory factor ofKirchmayer andSzczepkowski (KS factor) has a direct action of the PNH corpuscles themselves. Thrombin increases the haemolytic tendency of the erythrocytes by direct action. This is probably the result of enzyme action on the cell surface, similar to that observed byYachin, Laforet & Gardner in the case of normal erythrocytes. No effect of thrombin on the serum factors inhibiting haemolysis, as accepted byCrosby, could be detected.Kirchmayer andSzczepkowski had results similar to ours. The significance of coagulation factors in relation to PHN corpuscles in vivo is supported by the therapeutic effects of prolonged treatment with dicoumarol.
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Hoppe, I. Zur experimentellen Hämolyse von Erythrozyten bei der paroxysmalen nächtlichen Hämoglobinurie. Blut 8, 326–334 (1962). https://doi.org/10.1007/BF01630302
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DOI: https://doi.org/10.1007/BF01630302