title: [Hemostasis disorders after transfusions].
abstract: Disturbances of haemostasis caused immunologically and non-immunologically were observed after transfusion of blood and blood derivatives. Transfusion of heparin blood increased the bleeding susceptibility only in case of pre-existing high-degree defects of haemostasis or if they were performed as massive or exchange transfusions. Massive transfusions with blood stored for a long time will induce complex defects. Under intensive substitution therapy of haemophilia A the so-called paradoxical bleeding will occur in spite of a high factor VIII level. These bleedings are supposed to be disturbances of the thrombocyte function and are caused by fibrin(ogen) derivatives. Post-transfusional thrombocytopenias may be brought to remission by repeated plasmapheresis. Factor specific inhibitory bodies will appear after substitution in a small percentage of haemophilic patients. 5 to 7 days after the onset of therapy an anamnestic reaction can be observed as a titre increase by leaps. Usually, the inhibitory titre will decrease to a mostly low basal value in the course of three to five months. The therapy with cyclophosphamide simultaneously started with the substitution will more frequently prevent the anamnestic reaction or reduce it. Titres with more than 5 units cannot be overcome at the beginning even by higher concentrations of preparations. The substitution therapy should be preceded by exchange transfusions or plasmapheresis of up to 25 units. With still higher titres only procedures of inhibitor-bypassing are possible with factor VIII preparations of animal origin or better with activated prothrombin complex preparations, such as FEIBA. Recent reports give evidence that permanent substitution with factor VIII concentrates at a highest dosage can eliminate the production of inhibitors completely.
mesh_terms: D001778:Blood Coagulation Disorders / Q000276:immunology*; D001803:Blood Transfusion; D003520:Cyclophosphamide / Q000627:therapeutic use; D005164:Factor IX / Q000627:therapeutic use; D015949:Factor IXa; D005176:Factor XIII / Q000037:antagonists & inhibitors / Q000276:immunology; D005337:Fibrin / Q000032:analysis; D005340:Fibrinogen / Q000032:analysis; D006467:Hemophilia A / Q000276:immunology; D006487:Hemostasis; D006493:Heparin / Q000009:adverse effects; D006801:Humans; D010974:Platelet Aggregation / Q000187:drug effects; D013921:Thrombocytopenia / Q000276:immunology; D014842:von Willebrand Diseases / Q000276:immunology
chemical_list: D003520:Cyclophosphamide; D005164:Factor IX; D005337:Fibrin; D005340:Fibrinogen; D006493:Heparin; D005176:Factor XIII; D015949:Factor IXa
vernacular_title: Hämostasestörungen nach Transfusionen.