The up-dated case history of the index patient with the factor X Friuli coagulation disorder is reported. The patient, aged 72, died of irreversible shock after progressive jaundice with symptoms of renal, cardiac and hepatic failure. The autopsy showed carcinoma of the head of the pancreas, diffuse petecchial hemorrhages, massive adrenal gland hemorrhage and minimal athero-arteriosclerotic lesions of all arteries. The significance of minimal athero-arteriosclerotic changes in a patient with a congenital coagulation defect is discussed.

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