Depending on the presence of FVII antigen
(FVII:Ag) in the plasma, congenital FVII deficiency can be divided in type I
or type II. In type I, FVII:Ag deficiency results from either decreased
biosynthesis or accelerated clearance, whereas a dysfunctional FVII:Ag
characterizes type II. Generally, features may include hemarthros,
menorrhagia, hematuria, epistaxis, gingival bleeding, gastrointestinal
bleeding, retroperitoneal hematomas, and fatal cerebral hemorrhages or hematomas.
Clinically, the disease has been divided in four forms: (1) severe
life-threatening form (15% of patients) manifesting with neonatal
intracranial bleeding and often lethal course in infancy; (2) severe
hemorrhagic form (20%) with recurrent hemarthros and consequently chronic
arthropathy; (3) mild, late-onset form (60%) with postoperative cutaneous
or mucosal bleeding; and (4) asymptomatic form. Exclude acquired FVII
deficiency secondary to liver disease, vitamin K deficiency (warfarin
therapy, malabsorption), and, rarely, severe infections. Paradoxically, a
few patients develop thrombotic complications such as pulmonary embolus
and/or myocardial infarction.