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Abstract
The coagulation system was investigated in five patients with hemolytic diseases, three patients with paroxysmal nocturnal hemoglobinuria (PNH) and two with congenital spherocytic hemolytic anemia. A tendency to short cephalin times, and high levels of antihemophilic A factor (AHA = factor VIII) and fibrinogen was observed. The results indicate a tendency to a hypercoagulable state of the blood in patients with hemolytic diseases.
Experiments were done with injections of different amounts of sterile hemolysed own blood intravenously into normal persons, with investigations of plasma clotting system before arid after the injections. Injection of 30 ml hemolysed citrated own blood into two normal persons produced a temporary increase in the levels of AHA, fibrinogen and proaccelerin (factor V), with top values of AHA and fibrinogen on the second day after the injection.
The results of the injections of lysed blood in normal persons in the direction of hypercoagulability seem to he in agreement with the findings in the investigations of the patients with hemolytic diseases.
An effect of intravascular hemolysis on the coagulation system might be explained as a stimulation of the formation of AHA, fibrinogen and proaccelerin, possibly as an effect on the reticulo-endothelial system.