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 F1+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.

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Correspondence to W. Korte.

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Korte, W., Feldges, A. Increased prothrombin activation in a patient with congenital afibrinogenemia is reversible by fibrinogen substitution. Clin Investig 72, 396–398 (1994).

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Key words

  • Afibrinogenemia
  • Prothrombin activation fragments
  • Substitution therapy
  • Thrombin activation
  • D-dimer