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)

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

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

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.

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

Hemophilia Excessive subcutaneous bleeding after minor injury

Hemophilia Hemophilic person Subcutaneous bleeding after an injection prick

Transmission of Hemophilia from parents to children

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.

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.

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)

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

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

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

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

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