Blood to remain in effective circulation has to be in a fluid state, meaning it does not clot (any such eventuality can prove disastrous as in a myocardial infarction ‘heart attack’). At the same time, clot formation is essential to prevent unnecessary blood loss say after an injury or trauma. In short, both the process of maintaining a liquid sate of blood and formation of blood clot are a feature of healthy normal living.
Many disease states can affect any of these- abnormal clot formation or abnormal bleeding. These diseases can be acquired during lifetime or may be present since birth being hereditary or congenital, and may affect one of the many component of this intricately balanced system in human body:
- Vascular component
- Platelets, quantitative and qualitative defects
- Defects in the Coagulation proteins (Clotting factors)- there are more than a dozen!
- Excessive fibrinolysis
- Combined defects
Haemophilia is a type of bleeding disorder of hereditary or congenital nature, resulting from the deficiency of clotting factor VIII (Haemophilia A) or factor IX ( Haemophilia B). The disease is seen exclusively in males, however women do not clinically manifest this disease. These persons get bleeding from minimal trauma or even no trauma (spontaneous bleed). Bleeding can occur in any part of the body and become serious in case if critical organ like brain, chest or abdomen is involved.
Even though hemophilia has a genetic basis, there is a highly effective treatment with injection of clotting factors VIII or IX.
For effective treatment, exact and specific diagnosis of the deficient factor is must. Diagnosis of haemophilia in emergency situations is extremely difficult. There are preventive, protective and rehabilitative programmes for haemophilia.