2. Bleeding disorders
Deficiency of any of the clotting factors leads to excessive bleeding
Most common and important bleeding disorders are due
Vitamin K deficiency
Platelets deficiency (Thrombocytopenia)
Deficiency of Factor VIII (Hemophilia)

3. Hemophilia Genetic disorder
Deficiency of factor VIII– classical or type A
Factor IX– Hemophilia B or Christmas disease
Chromosome X linked recessive disorder
Females carrier & males are diseased
Factor VIII has 2 components
↓ Small component → classical hemophilia
↓ large component → von Willebrand's disease
Treatment
Injection of purified factor VIII – very expensive
Frequent blood transfusions

4. Symptoms of Hemophilia A
Bruising
Bleeding into joints with pain and swelling
Spontaneous bleeding
Gastrointestinal tract and urinary tract hemorrhage
Prolonged bleeding
Blood in the urine or stool

5. Hemophilia B Also known as Christmas disease.
Also inherited blood clotting disease caused by a deficiency factor IX.
Hemophilia A is seven times more common than hemophilia B.

6. Symptoms of Hemophilia B
Nose bleeds
Bruising
Spontaneous bleeding
Bleeding in joints
Blood in urine or stool
Prolonged bleeding from cuts, tooth extraction, and surgery
Excessive bleeding following circumcision

7. Hemophilia
Excessive subcutaneous bleeding after minor injury

8. Hemophilia Hemophilic person
Subcutaneous bleeding after an injection prick

9. Transmission of Hemophilia from parents to children

10. Both of these factors(8,9) are transmitted genetically by way of the female chromosome. Therefore, almost never will a woman have hemophilia because at least one of her two X chromosomes will have the appropriate genes. If one of her X chromosomes is deficient, she will be a hemophilia carrier, transmitting the disease to half of her male offspring and transmitting the carrier state to half of her female offsprings.

11. Mother is a carrier, father is normal: XX. XY Offspring: XX XY X. X X
Mother is a carrier, father is normal: XX. XY Offspring: XX XY X.X X.Y 50% of her female offsprings are carriers, 50% of her male offsprings are diseased.

12. Anticoagulants Anticoagulant: prevents clots. Heparin - stops thrombin
Recombinant tPA (tissue plasminogen activator), greatly lessens impact of heart attacks
Coumarin drugs (dicumarol, warfarin)
Inactivate Vit. K resulting in reduction of many clotting factors
Na-citrate, Na-oxalate, K-citrate, K-oxalate, EDTA (Ca++ removal)

13. Heparin 0. 5 – 1 mg/kg body weight
Heparin 0.5 – 1 mg/kg body weight. - Increases clotting time from 6 min to 30 min or more - Prevents further development of thromboembolic condition - Action of heparin lasts 1.5 – 4 hours - Heparin is destroyed by an enzyme heparinase

14. Coumarins (Warfarin) - Warfarin has a potent depressant effect on liver formation of 7, 9, 10 - Warfarin competes with vitamin K for formation of prothrombin and other 3 clotting factors - Coagulant activity decreases to about 50% of normal by the end of 12 hours and to about 20% of normal by the end of 24 hours - Normal coagulation returns 1 to 3 days after discontinuing coumarin therapy

15. Thromboembolic conditions
Abnormal clot in blood vessels- thrombus
Clot detached from thrombus – Embolus
Emboli may originate in the heart
Emboli may block any blood vessels any where in the body
Emboli from the veins usually cause pulmonary artery embolism

16. Causes of thromboembolism
Roughened endothelial surface
Arteriosclerosis
Infection
Trauma
Sluggish blood flow
Prolonged rest or immobility
Diseases leading to inactivity for long time
e.g. Pulmonary embolism from thrombus in the veins of the legs

17. Thank-you
Questions ??