A 65-year-old man was admitted to another hospital with a life-threatening
brain haemorrhage, and laboratory examinations on admission revealed prolon
ged prothrombin time with normal activated partial thromboplastin time. To
establish the cause of his abnormal coagulation, he was referred to our cli
nic. Neither the patient nor his family had any previous history of bleedin
g symptoms. His liver function was within normal limits but coagulation tes
ts showed increased plasma activities of factors II, VIII, IX, X, with redu
ced activities of factors V and VII. The activity of factor VII was less th
an 2% but no inhibitor of factor VII was detected in the plasma. We conclud
ed that the patient had a rare congenital isolated factor VII deficiency al
though. he had not shown earlier bleeding problems, presumably because of c
ompensation for the factor VII deficiency by enhanced activities of compone
nts of the extrinsic coagulation pathway, factors II, VIII, IX and X.