[A case of Osler-Rendu disease with simultaneous thrombopenia and a factor VIII inhibitor].
Long-term determinations of haemostasis factors in a case of Osler's telangiectasia revealed the temporarily simultaneous existance of periods of thrombocytopenia, a decrease of prothrombine and a reduction of the fibrinogen and plasminogen level. These findings considered as signs of consumption coagulopathy coincided with an increased bleeding tendency of the patient. The correlation existing between the clinical symptoms and the observed disorders of coagulation may possibly be explained by the appearance of an intravascular coagulation during the late period of the haemorrhagic diathesis, which could be proved by the simultaneous increase of fibrinogen degradation products. Moreover, the patient's plasma was capable of strongly inhibiting factor VIII of a normal plasma. The possible influence of plasmatic disorders of coagulation which are caused by secondary reasons on the clinical picture of a haemorrhagic diathesis primarily based on vascular conditions is discussed.
D004697:Endocarditis, Bacterial / Q000209:etiology; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D006801:Humans; D008297:Male; D008581:Meningitis / Q000209:etiology; D008875:Middle Aged; D010375:Pedigree; D018805:Sepsis / Q000209:etiology; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000235:genetics; D013921:Thrombocytopenia / Q000150:complications*
D005169:Factor VIII
Ein Fall von Morbus Osler mit gleichzeitig bestehender Thrombozytopenie und einem Faktor VIII-Inhibitor.