Severe Factor X Deficiency-A Rare Coagulation Disorder

Factor X is a vitamin K-dependent factor with pivotal role in as the first enzyme in the common pathway to fibrin formation. Inherited factor X deficiency is a rare bleeding disorder. Based on the Factor X coagulant activity the disorder has been divided into severe (<1%), moderate (1–5%) and mild (6–10%). Levels above 20% are infrequently associated with bleeding and heterozygotes are usually asymptomatic. Among patients with mild Factor X coagulant activity, majority of them present with mucocutaneous bleeding symptoms such as epistaxis and menorrhagia. In addition, patients with moderate to severe deficiency may have symptoms similar to that of haemophilia A and B. While no purified FX concentrate is commercially available, haemostasis can be secured using fresh-frozen plasma (FFP) or prothrombin complex concentrates (PCC).