Increased prothrombin activation in a patient with congenital afibrinogenemia is reversible by fibrinogen substitution.
We describe a patient with congenital afibrinogenemia who showed elevated prothrombin activation fragments (F1 + 2) indicating increased thrombin formation. This finding was unexpected since it has hitherto been thought that patients with congenital hypo- or afibrinogenemia have no evidence of increased utilization or accelerated consumption of coagulation factors. No other possible reasons for the elevation of F 1 + 2 were found. Upon fibrinogen substitution F1 + 2 decreased and were again increasing when fibrinogen concentration in plasma fell to very low levels. These findings raise the question of whether increased thrombin formation should be understood as a compensatory mechanism in congenital afibrinogenemia.
Korte
W
W
Institut für Klinische Chemie und Hämatologie, Kantonsspital, St. Gallen, Schweiz.
Feldges
A
A
D000293:Adolescent; D000347:Afibrinogenemia / Q000097:blood* / Q000188:drug therapy; D004789:Enzyme Activation; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D005340:Fibrinogen / Q000627:therapeutic use*; D006801:Humans; D010446:Peptide Fragments / Q000032:analysis; D011516:Prothrombin / Q000032:analysis / Q000378:metabolism*
D005338:Fibrin Fibrinogen Degradation Products; D010446:Peptide Fragments; C036309:fibrin fragment D; C045879:prothrombin fragment 1.2; D011516:Prothrombin; D005340:Fibrinogen