Defibrination syndrome in a patient with chronic thrombocytopenic purpura

The clinical course and laboratory findings are described in a patient with prolonged defibrination syndrome. The patient had had recurrent thrombocytopenia for twenty-five years, and lupus erythematosus cells, previously demonstrable, were not found on the present admission. The present admission was because of purpura and thrombocytopenia associated with hypofibrinogenemia. The occurrence of bilateral femoral vein thromboses indicated intravascular coagulation, and grossly elevated fibrinolytic split product levels indicated excessive fibrinolysis. The administration of heparin corrected defibrination and reduced fibrinolytic split product levels to normal. It was inferred that defibrination primarily resulted from coagulation, that excessive fibrinolysis was secondary to intravascular coagulation and that heparin inhibited fibrinolysis primarily by inhibiting coagulation. During a one year follow-up the patient had partial thromboplastin clotting times consistently shorter than normal and stable high levels of factors VIII(mean 246 per cent), XI (mean 181 per cent), X (mean 145 per cent), V (mean 183 per cent) and XII (mean 171 per cent). During defibrination one-stage factor XI levels (mean 220 per cent) were con sistently higher than two-stage levels (mean 102 per cent), whereas the two methods gave similar results during the follow-up period. It is suggested that the discrepant results reflect the presence of activated coagulation factors; the significance of this finding is discussed. As compared with the follow-up period, defibrination factor X levels were slightly elevated, two-stage factor XI levels reduced and factor IX levels elevated. The in vivo changes do not resemble the pattern of changes resulting from activation of the intrinsic or extrinsic systems in vitro.