Inherited dysfibrinogenaemia presenting with portal vein thrombosis.

A 15 years old female presented with recurrent episodes of gum bleeding, easy bruisibility and recurrent soft tissue haematoma following trivial trauma since childhood. Subsequently the patient developed features of extrahepatic portal vein obstruction due to formation of thrombus. Defective fibrinogen structure due to underlying inherited dysfibrinogenaemia led to the manifestations of both bleeding due to coagulation failure and thrombosis due to failure of abnormal fibrin to get lysed.