In a family with 14 children severe deficiency of factor XIII was found in three living members who suffered from moderate to severe bleeding manifestations. A fourth child died with intracranial hemorrhage. In addition to the above deficiency, two of the three propositi and two siblings were mildly deficient in factor XII. The bleeding manifestations of these cases are solely due to factor XIII deficiency; siblings with factor XII deficiency alone were asymptomatic. Both factor deficiencies here seem to be separately inherited.
The most striking findings in factor XIII deficiency are bleeding from the umbilical cord, which is invariably found, and intracranial hemorrhage frequently encountered in the pediatric age group. The present communication, for the first time, reports on gastrointestinal hemorrhage as a manifestation of this disease. Also, in this family hemarthrosis was not uncommon and characteristically did not lead to residual crippling.