COAGULATION AND FIBRINOLYTIC STUDIES INIPAROXYSMAL NOCTURNAL HAEMOGLOBINURIA

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Abstract
Seven patients with paroxysmal nocturnal haemoglobinuria (PNH) have been examined during quiescent haemolysis as well as during haemolytic exacerbations with thrombotic manifestations. In six of the patients hypercoagulability was observed as indicated by short kaolin‐cephalin time, accelerated thromboplastin generation screening test and/or increased activity of factors II + VII and VIII. In three cases it was demonstrated that the patient plasma could accelerate the thromboplastin generation of normal plasma. No abnormalities were observed in fibrinolysis, nor changes indicative of consumption coagulopathy. The significance of the demonstrated hypercoagulability is unknown. The conclusion of our findings must therefore be limited to the observation that in PNH patients a certain “laboratory hypercoagulable state” can be demonstrated and that this “hypercoagulability” increases during the haemolytic crises.