Hereditary Factor VII deficiency in 17 Iraqi patients

Abstract

Summary:Background: The objective of this study is to (1) determine the most common presenting and frequent hemorrhagic symptoms, in Iraqi patients suffering from F.VI1 deficiency, (2) The figure of prothrombin time ratio that make the physician suspect hereditary F. VII deficiency. Patients and Methods: This is a retrospective study conducted by reviewing the records of 760 patients registered in the center of congenital coagulation disorder in Al.mansour teaching hospital.All patients who diagnosed as factor VII deficiency were included in this study. The diagnosis was based on prolonged PT with normal PTT and bleeding time. Clinical information regarding age, presenting features and blood group were also considered.Results: out of 760 patient registered in the center of congenital coagulation disorders, 17 patients were considered as having F. VII deficiency: they constitutes 2.2% of all recorded patients.The most common presenting feature was haemarthrosis (37.5 % ) followed by intracranial bleeding (18.7%) and ecchymosis (18.7 %) .The most common haemorrhagic symptom recorded was haemarthrosis (50%), epistaxis (50%) followed by ecchymosis (43.7%) .Patients with in intracranial bleeding constitute 25%. The majority of patients (88.2%) have prothrombin time ratio of more than 3.5Conclusion : Hereditary F. VII deficiency should be suspected in :1-Children presented with haemarthrosis, intracranial bleeding, epistaxis or ecchymosis2-Children show prothrombin time ratio of 1.3 and more with normal PTT and normal bleeding time.Keyword: Hereditary F. VII deficiency , Prothrombin time ratio.