[Alteration of platelet function during intensive replacement therapy in haemophilia A (author's transl)].
This report describes two patients with haemophilia A who developed a transient thrombocytopathy with haemorrhagic diathesis during post-operative high-dose replacement therapy with antihaemophilic globulin. At the time of the bleeding the factor VIII-activity was in the normal range in both patients. The fibrinogen level, however, was elevated to 1700 mg-% and the factor VIII-associated antigen rose to more than 6-fold. At no time of replacement therapy with antihaemophilic globulin could either fibrinogen split products or fragments of the factor VIII-protein be detected by the usual methods. In view of the results of the thrombocyte aggregation experiments the authors postulate a disturbance of platelet function at the level of the membrane surface due to an overload of increased amounts of circulating proteins. Both the possible interference of dialysable factor VIII-components and the role of immunpathologic phenomena are discussed.
D000328:Adult; D000941:Antigens / Q000032:analysis; D001791:Blood Platelet Disorders / Q000097:blood / Q000209:etiology*; D002648:Child; D005169:Factor VIII / Q000009:adverse effects* / Q000032:analysis / Q000276:immunology / Q000627:therapeutic use; D005340:Fibrinogen / Q000032:analysis; D006467:Hemophilia A / Q000097:blood / Q000628:therapy*; D006474:Hemorrhagic Disorders / Q000097:blood / Q000209:etiology*; D006801:Humans; D066298:In Vitro Techniques; D008297:Male; D010974:Platelet Aggregation
D000941:Antigens; D005169:Factor VIII; D005340:Fibrinogen
Thrombozytenfunktionsstörung während der hochdosierten Substitutionstherapie bei Hämophilie A.