Haemophilia

 

What Is Haemophilia?

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Haemophilia is an inherited hemorrhagic blood disorder. The defect lies in the clotting mechanism of the blood and is characterised by a life long tendency to excessive internal or external hemorrhage.

This blood disorder can be classified into three kinds, Haemophilia ‘A’ is due to the deficiency of factor VIII. Haemophilia ‘B’ also known as Christmas disease is due to the deficiency of factor IX. The third kind ‘C’ is due to the deficiency of factor XI. One more related disease called Von Willie Brand’s is due to the deficiency of VW antigen deficiency.

Patients with Haemophilia very often have internal or external bleeding. It can be spontaneous or can occur on trauma. Symptoms include gum bleeding, bleeding from nose, bleeding in large joints of the elbows, knees, ankles and shoulder joints, bleeding in urine, bleeding in stool and bleeding in the brain. The bleeding mostly occurs in the large joints.

If Haemophilic patients remain untreated, the result would be prolonged excessive bleeding which may prove fatal and cause severe anaemia. Bleeding in joints causes severe deformities of joints, flexion/extension deformities, weakness of legs, shortening of leg that can lead to crippling. Bleeding in the brain and abdomen are very dangerous and may cause death.