Factor VII Deficiency Presenting with Recurrent Intracranial Bleeding and rFVIIa Prophylaxis.

Abstract

Factor VII deficiency is an autosomal recessively inherited bleeding disease with a frequency of 1/500000. Intracranial bleeding may be seen in the newborn and infancy period. In this report, a patient having reccurrent intracranial bleedings with the diagnosis of Factor VII deficiency that is treated and given the prophylaxis of rFVIIa is discussed. The patient was a 44 months old boy who was given fresh frozen plasma at the 2 and 4 months of age. He had subdural hematoma, intracranial bleeding and hydrocephalus when he was 5 months of age and ventriculoperitoneal shunt operation was done. Replacement therapy with rFVIIa was given. When he was 11 months of age he had 2 attacks of intracranial bleeding and right cerebellar hematoma was diagnosed at that time. He was treated with 14 days of rFVIIa replacement therapy, without the need of any surgical intervention. In the 15th month he had a 3rd attack of intracranial bleeding and after that bleeding he had left occipital lobe hematoma. The valve of his shunt was replaced. He was treated with 14 days of rFVIIa replacement therapy. During 15–26 months of age the patient was given fresh frozen plasma twice a week for 6 months and then continued with 10 ml/kg of fresh frozen plasma once a week. He had 4 minor mucosal bleeding attack. At the 26th month he had the 4th intracranial bleeding attack and subarachnoid hemorrhage that is treated with 14 days of rFVIIa replacement therapy. No surgical intervention was done.After the 4th attack, once a week rFVIIa prophylaxis was started. He had 4 minor mucosal bleeding during the prophylaxis. At the 34th month due to his social security problems the patient could not get the drug and he had the 5th bleeding and had subarachnoid bleeding in the 35th month. Both replacement therapy and prophylaxis were restarted. The patient is being followed for 7 months with rFVIIa prophylaxis and he had no new bleeding episode.