Summary
This report discusses congenital dysfibrinogenemia with facultative hemorrhagic diathesis
in a 27-year-old woman patient. The autosomal-dominant hereditary transmission is
assumed because of the occurrence of dysfibrinogenemia in the patient’s daughter now
two-years-old. More extensive genetic examinations were impossible as the patient
had been adopted.
The thrombin-time, the thromboplastin- and the partial thromboplastin-time were all
prolonged. Reptilase and arvin did not lead to visible coagulation. Immunologically
the concentration of fibrinogen was found to be normal, but fibrinogen, when determined
as a thrombin clottable protein was clearly reduced. The patient’s plasma and isolated
fibrinogen had an inhibitory effect on the coagulation of normal plasma or fibrinogen.
Inhibitory factors of the fibrinogen-fibrin transition or an increased fibrinolysis
was not found. An isolated determination of coagulation factors gave normal results.
The fibrin polymerisation of the patient’s isolated fibrin monomer was delayed and
did not reach the normal maximum. By applying bidimen- sional paper electrophoresis
to the patient’s isolated fibrinogen after incubation with thrombin the separation
of fibrinopeptide A was shown to be absent. Plasmin applied to the pathological fibrinogen
did not release any late lysis products. Based on these findings which in part differ
from other reported anomalies of fibrinogen, we propose to call this variant type
of fibrinogen Giessen.
