A 54-year-old man was brought to the emergency room after a head-on collision. He had
multiple fractures in his lower extremities and required immediate surgery. After surgery, the patient had a persistent drop in
hemoglobin, hematocrit and platelets despite
red blood cell transfusions. Laboratory studies included normal prothrombin time, activated partial thromboplastin time, normal
plasminogen functional activity, negative antiplatelet
antibodies, normal platelet functional analysis and negative
disseminated intravascular coagulation screen.
Factor XIII antigen levels were 25% of predicted, and the diagnosis of
factor XIII deficiency was made. The patient was treated with cryoprecipitate, and the
bleeding stopped. Patients with
factor XIII deficiency have either a rare congenital or acquired coagulation disorder. Both presentations have normal standard laboratory clotting tests, and the diagnosis requires an assay measuring
factor XIII activity or
antigen levels. The usual treatment includes cryoprecipitate, fresh-frozen plasma or recombinant
factor XIII. This deficiency should be considered in patients with unexplained spontaneous, traumatic or postoperative
bleeding.