Factor 13 Deficiency with Severe Hemorrhagic Diathesis

Abstract

Abstract This report presents the first case of Factor 13 deficiency in an adult female. Her hemorrhagic manifestations were repeated and severe: umbilical vein bleeding at birth, hematomas on various sites, late bleeding after cuts and, most important, severe uterine bleedings during 12 pregnancies, which were all interrupted by spontaneous abortions. Plasma infusions, assuring a Factor 13 concentration of less than 10 per cent of its normal level, normalized the hemostatic functions of the patient’s plasma clots and rendered them insoluble in concentrated urea solutions. By regular transfusions of plasma the patient’s last pregnancy had a normal course; a cesarian section was performed without any incident, and she gave birth to a normal child.