A marked abnormality of the intrinsic coagulation system was observed in a patient with melanomatosis and frank melanuria. With successful treatment of the tumour there was a parallel improvement in the clotting abnormality. This defect was shown to be a deficiency of factor XI together with a previously unrecognised factor. This is distinct from either Fletcher or Fitzgerald factors and appears to act between factors XI and X in the coagulation sequence.
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