Thrombin Generation and Clot Retraction Studies in Paroxysmal Nocturnal Hemoglobinuria

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Abstract
Generation of thrombin in platelet-rich and platelet-free plasma of 4 patients with paroxysmal nocturnal hemoglobinuria (PNH) was demonstrated to be the same as in normal persons. There was the normally occurring difference in the amount of thrombin generated in each of these 2 compartments, the results being related to the platelet concentration. The ability of PNH platelets to promote clot retraction was normal in all 4 patients studied. This investigation provides no evidence for the hypothesis that by virtue of a stromal defect similar to that of the PNH erythrocyte, the PNH platelet is lysed by the same plasma system that hemolyzes the red cells.