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Health

1. BLEEDING DISSODERS Dr. Tilak Abeysekera MD, MRCP Consultant physician [email protected]

2. Haemostasis 1. vascular endothelium 2. platelets 3. coagulation factors 4. natural anticoagulants 5. fibrinolytic enzymes

3. Anti platelets drug Cyclo oxygenase inhibitors – aspirin Inhibitors of Adenosine DiPhosphate (ADP) mediated activation – clopidogrel

4. Clotting factors

5. Clotting factors Synthesis by the liver Factor 5 produced by platelets and endothelial cells Vitamin K is required for clotting factor synthesis Warfarin interfere with clotting factor formation

6. Investigations of coagulation

7. Monitoring of anti coagulant therapy International Normalized Ratio (INR) INR is the ratio of the patient’s prothrombin time to that of a normal control, raised to the power of the international sensitivity index of the thromboplastin used in the test.

8. Vessel wall abnormalities Congenital – hereditary hemorrhagic telangiectasia Acquired – vasculitis, scurvy

9. Platelets functional disorders

10. Thrombocytopenia Decreased production

11. Increased consumption of platelets

12. Idiopathic thrombocytopenic purpura (ITP) Can affect any age group First line therapy for patients with spontaneous bleeding is prednisolone

13. Coagulation disorders

14. Coagulation disorders Hemophilia A Hemophilia B (Christmas Disease) Von Willebrand disease

15. Acquired bleeding disorders Liver disease Renal failure

16. Hemophilia A Hemophilia A is due to Factor VIII deficiency It is the most common congenital factor deficiency Factor VIII is synthesized by the liver and endothelial cells Factor VIII gene is located in the X chromosome. Therefore hemophilia A is a sex linked disorder

17. Clinical features

18. Management IV infusion of factor VIII concentrate

19. Thank You !