Severe congenital factor X deficiency – An unusual cause of intracranial hemorrhage

Abstract

Factor X deficiency is one of the rare inherited coagulation disorders. Symptoms vary from mild to severe with the commonest being epistaxis, gum bleeds and menorrhagia. The diagnosis is suspected when both PT and aPTT are prolonged and remains uncorrected following Vitamin K administration. Replacement of Factor X can be accomplished with Fresh Frozen Plasma(FFP) or plasma derived concentrates such as Prothrombin Complex Concentrates(PCC). We report a male child who presented with intracranial haemorrhage at age of 18 months as the presenting manifestation of severe congenital Factor X deficiency.

Keywords

• clotting factor deficiency
• subdural hematoma
• fresh frozen plasma
• prothrombin complex concentrate