Summary
This report describes two patients with haemophilia A who developed a transient thrombocytopathy with haemorrhagic diathesis during post-operative high-dose replacement therapy with antihaemophilic globulin. At the time of the bleeding the factor VIII-activity was in the normal range in both patients. The fibrinogen level, however, was elevated to 1700 mg-% and the factor VIII-associated antigen rose to more than 6-fold. At no time of replacement therapy with antihaemophilic globulin could either fibrinogen split products or fragments of the factor VIII-protein be detected by the usual methods. In view of the results of the thrombocyte aggregation experiments the authors postulate a disturbance of platelet function at the level of the membrane surface due to an overload of increased amounts of circulating proteins. Both the possible interference of dialysable factor VIII-components and the role of immunpathologic phenomena are discussed.
Zusammenfassung
Es wird über 2 Patienten mit Hämophilie A berichtet, bei denen sich während einer operationsbedingten hochdosierten Substitutionstherapie mit antihämophilem Globulin vorübergehend eine Thrombozytenfunktionsstörung mit manifester hämorrhagischer Diathese einstellte. Zum Zeitpunkt der Blutung waren bei ausreichend hoher Faktor VIII-Aktivität der Fibrinogenwert im Plasma auf 1700 mg-% und der Gehalt an Faktor VIII-assoziiertem Antigen auf über 600% der Norm angestiegen. Da es während der Substitution zu keiner Zeit gelang, Fibrinogenspaltprodukte oder Komponenten des antihämophilen Globulins mit üblichen Methoden nachzuweisen, wird — unterstützt durch „in vitro“-Untersuchungen der Thrombozytenaggregation — die Ansicht vertreten, daß die Beladung der Plättchenoberfläche mit überschüssig zugeführten Proteinen ursächlich mit der thrombozytären Funktionsstörung zusammenhängt. Die Bedeutung kleinmolekularer und dialysierbarer Faktor VIII-Komponenten wird ebenso wie die Möglichkeit immunpathologischer Vorgänge diskutiert.
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Gadner, H., Odenwald, E., Jarofke, R. et al. Thrombozytenfunktionsstörung während der hochdosierten Substitutionstherapie bei Hämophilie A. Klin Wochenschr 55, 1165–1171 (1977). https://doi.org/10.1007/BF01478054
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DOI: https://doi.org/10.1007/BF01478054