Congenital Hemorrhagic Diathesis with Deficiency of Factor XIII

Abstract

SummaryA male patient with a severe congenital bleeding disorder due to factor XIII deficiency is described. An extensive family study revealed no additional demonstrable deficiencies of this factor. A lowered fibrinogen level in four of the father’s relatives was found, and remained unexplained. Prophylactic treatment with plasma transfusions appeared to be effective.