Congenitale deficiëntie van de fibrinolyseremmer [alpha]2-antiplasmine als oorzaak van hemorragische diathese

– In this article the clinical importance of α₂-antiplasmin is demonstrated by presenting the history of a male aged 19 years suffering from α₂-antiplasmin deficiency. In the literature 11 families with congenital α₂-antiplasmin deficient members are reported, of which 5 families are Dutch. Information is given about the severe haemorrhagic tendency in these patients, and the difference from haemophilia is described in detail. Congenital α₂-antiplasmin deficiency can be diagnosed with a simple enzymatic assay. Bleeding problems associated with α₂-antiplasmin deficiency can be treated with fibrinolytic inhibitors such as epsilon aminocaproic acid and tranexamic acid (Cyklokapron).