Abstract
A family with hereditary autosomal dominant hypofibrinogenemia is described. The outstanding feature is massive deposition of fibrinogen/fibrin within hepatocytes, faintly visible in routine microscopic sections, but clearly demonstrable by immunohistologic techniques. Circulating fibrinogen shows normal electrophoretic mobility of Aα-, Bβ-, and γ-chains. We assume that the hereditary defect in this family interferes with fibrinogen release from hepatocytes. Clinically there are fluctuating slight elevations of serum transaminase levels. Hemostasis and wound healing are undisturbed.
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Wehinger, H., Klinge, O., Alexandrakis, E. et al. Hereditary hypofibrinogenemia with fibrinogen storage in the liver. Eur J Pediatr 141, 109–112 (1983). https://doi.org/10.1007/BF00496800
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DOI: https://doi.org/10.1007/BF00496800