Case report of an acquired factor XIII inhibitor: diagnosis and management.

摘要: A 57-year-old man presented with a spontaneous upper-extremity hematoma and compartment syndrome. The patient experienced excessive bleeding following evacuation of the hematoma, results routine coagulation studies were normal. Factor XIII activity was undetectable using photometric assay, presence an inhibitor detected mixing studies. Bleeding controlled infusions fresh frozen plasma cryoprecipitate. Cyclophosphamide started on 16th hospital day, four weekly doses monoclonal anti-CD20 antibody, rituximab, begun 3 weeks later. One week after initial dose no longer detectable factor level increased to 28%. After completion rituximab therapy, 58% present. This case illustrates need check for unusual defects such as deficiency if tendency is evident—even are unrevealing.