GENETIC DEFICIENCY OF FACTOR-VII AND HEMORRHAGIC DIATHESIS - A CASE-REPORT AND LITERATURE-REVIEW

FVII deficiency is a rather rare inherited hemocoagulation disorder th at predisposes to hemorrhagic events, especially from mucous membranes , that are not predictable and severe as in hemophilia A. This defect produces prolonged prothrombin time (PT), reduced activity of FM and n ormal activated partial thromboplastin time (aPTT). We report the case of a 43-year-old obese woman with severe deficiency of factor VII (FV II), probably genetic in nature, and meno-metrorrhagia associated with multiple fibromas of uterus. Our patient had no history of bleeding i n infancy and young age, and in the past, before the disease was diagn osed, underwent major surgery operations (thyroidectomy and caesarian section) without hemorrhage. Patient's relatives with mild heterozygou s deficiency of FVII (the father, a brother, a sister, a sister's daug hter and the patient's son) did not show any bleeding tendency. This c ase report is discussed in the light of literature data (source: Medli ne from 1964 to 1996). The different forms of congenital (isolated or combined with other clotting disorders) and acquired FVII deficiency, with the appropriate therapies, are reviewed. The clinician must consi der FVII deficiency in cases of recurrent bleeding, and this disease, even if rather rare, should not be underestimated in clinical practice because it is potentially fatal.