[Congenital deficiencies of coagulation factors and acquired inhibitors leading to bleeding disorders].
This review focuses on symptoms, course and treatment of bleeding disorders due to hereditary coagulation factor deficiencies and acquired inhibitors, mentioning as well the pathophysiologic and molecular genetic aspects and diagnostic particularities. The review of haemophilia A and B deals with carrier problems, replacement therapy, additional haemostatic agents such as antifibrinolytics and desmopressin, the treatment of typical haemorrhages, haemophilia with inhibitors and future therapeutic options. Of the autosomal homozygous bleeding disorders such as von Willebrand disease type 3, afibrinogenaemia, factor XIII-, VII- and XI-deficiency each has its particularities influencing treatment strategies. The last chapter discusses acquired bleeding disorders such as acquired haemophilia A, von Willebrand disease, factor V deficiency and the hypoprothrombinaemia lupus anticoagulant syndrome, the different modes of inhibition, diagnostics and principles of treatment.
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Frohbergstrasse 7, 8620 Wetzikon, Schweiz.
D001778:Blood Coagulation Disorders / Q000097:blood / Q000235:genetics* / Q000628:therapy; D006467:Hemophilia A / Q000235:genetics / Q000628:therapy; D006470:Hemorrhage / Q000235:genetics / Q000628:therapy; D006801:Humans; D014842:von Willebrand Diseases / Q000235:genetics / Q000628:therapy