Hereditary factor VII deficiency in two siblings: two different clinical presentation.

Factor VII (FVII) deficiency is a rare bleeding disorder with a highly variable hemorrhagic predisposition. It is transmitted as an autosomal recessive and plays an important role in the initiation of blood coagulation forming a complex with tissue factor which activates FIX, FX and FVII zymogen. Prolonged prothrombin time with normal partial thromboplastin time indicates FVII deficiency. For the definitive diagnosis, the specific plasma FVII level should be investigated. FVII deficiency is expressed in different ways and leads to various clinical pictures. I reported two siblings with hereditary factor VII deficiency whose clinical presentations were different.