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A 47-year-old man with congenital factor XIII deficiency developed recurrent hemarthrosis of the right ankle. He had radiologic evidence of permanent joint damage. A review of the literature revealed that hemarthrosis is not a rare manifestation of factor XIII deficiency; however, destructive changes in the joints are relatively uncommon in this disorder, in contrast with those changes seen in patients with classic hemophilia and arthropathy. The bleeding manifestations of factor XIII deficiency are readily correctable by plasma or cryoprecipitate infusions; this may account for the preservation of joint function and structure.
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