DOI: 10.36347/sjmcr.2023.v11i11.009

Pages: 1952-1955

Factor VII deficiency considered the most common of rare bleeding disorders. The symptoms of this affection can begin at any age, although the most severe cases are apparent in infancy. However, up to one-third of people with factor VII deficiency never have any bleeding problems. Symptoms can range from epistaxis up to cerebral hemorrhage. We here report the case of a child, diagnosed with factor VII deficiency with severe recurrent Bleedings (umbilical haemorrhage, hemarthrosis. lingual hematoma, gastrointestinal and cerebral bleeding). Prophylaxis with rFVIIa in a schedule based on 20-30 μg/kg of rFVIIa one, twice or three times a week has been described as the therapeutic regimen with the best outcomes in terms of reduction of bleeding's severity and frequency. Our patient underwent early prophylaxis with a 30 μg/kg of rFVIIa a week schedule. Unfortunately, prophylaxis hasen't been successful to prevent further bleedings. The evolution was marked by occurrence of several gastrointestinal and cerebral bleeding, then death by a cerebral hemorrhage at the age of 6 months. Congenital Factor VII deficiency is a rare cause of bleeding disorder, which should be suspected in a healthy bleeding child presenting in infancy when platelets and aPTT is normal with a deranged PT.