Abstract
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.
References
Bauer KA, Rosenberg RD (1987) The pathophysiology of the prethrombotic state in humans: insights gained from studies using markers of hemostatic system activation. Blood 70:343–350
Bruhn HD, Conrad J, Mannucci M, Monteagudo J, Pelzer H, Reverter JC, Samama M, Tripodi A, Wagner C (1992) Multicentric evaluation of a new assay for prothrombin fragments F1+2 determination. Thromb Haemost 68:413–417
Cadroy Y, Pierrejean D, Fontan B, Sie P, Boneu B (1992) Influence of aging on the activity of the hemostatic system: prothrombin fragments F 1+2, thrombin-antithrombin III complexes and D-dimers in 80 healthy subjects with age ranging from 20 to 94 years. Nouv Rev Fr Hematol 34:43–46
Caine YG, Bauer KA, Barzegar S, ten Cate H, Sacks FM, Walsh BW, Schiff I, Rosenberg RD (1992) Coagulation activation following estrogen administration to postmenopausal women. Thromb Haemost 68:392–395
Gralnick HR (1990) Congenital abnormalities of fibrinogen. In: Williams JW, Beutler E, Erslev AJ, Lichtman MA (eds) Hematology. McGraw-Hill, New York, pp 1474–1490
Torbet J (1986) Fibrin assembly in human plasma and fibrinogen/albumin mixtures. Biochemistry 25:5309–5314
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Korte, W., Feldges, A. Increased prothrombin activation in a patient with congenital afibrinogenemia is reversible by fibrinogen substitution. Clin Investig 72, 396–398 (1994). https://doi.org/10.1007/BF00252836
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00252836