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Bleeding disorder

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Priyanka Srivastava

Discussed the conditions which leads to excessive bleeding in Humans.includes Hemophilia, Thrombocytopenia,DIC and vitamin k deficiency.

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DR.PRIYANKA SRIVASTAVA
Excessive bleeding can result from deficiency of any one of the many blood-clotting factors. Three particular types of bleeding tendencies that have been studied to the greatest extent are discussed here: bleeding caused by (1) Vitamin K deficiency, (2) Hemophilia, and (3) Thrombocytopenia (platelet deficiency). Conditions which causes Excessive bleeding in Humans
Decreased Prothrombin, Factor VII, Factor IX, and Factor X Caused by Vitamin K Deficiency. With few exceptions, almost all the blood-clotting factors are formed by the liver. Therefore, diseases of the liver such as Hepatitis, Cirrhosis, and Acute yellow atrophy Depress the clotting system so greatly that the patient develops a severe tendency to bleed. Vitamin K Deficiency
Another cause of depressed formation of clotting factors by the liver is vitamin K deficiency. Vitamin K is an essential factor to a liver carboxylase that adds a carboxyl group to glutamic acid residues on five of the important clotting factors: Prothrombin, Factor VII, Factor IX, Factor X, and Protein C. Vitamin K Deficiency
In adding the carboxyl group to glutamic acid residues on the immature clotting factors, vitamin K is oxidized and becomes inactive. Another enzyme, vitamin K epoxide reductase complex 1 (VKOR c1), reduces vitamin K back to its active form. In the absence of active vitamin K, subsequent insuf- ficiency of these coagulation factors in the blood can lead to serious bleeding tendencies. Vitamin K Deficiency
Bleeding disorder
Bleeding disorder
Hemophilia is a bleeding disease that occurs almost exclusively in males. In 85% of cases, it is caused by an abnormality or deficiency of Factor VIII; this type of hemophilia is called hemophilia A or classic hemophilia. In the other 15% of hemophilia patients, the bleeding tendency is caused by deficiency of Factor IX. Both of these factors are transmitted genetically by way of the female chromosome. HEMOPHILIA
Females will be a hemophilia carrier, transmitting the disease to half of her male offspring and transmitting the carrier state to half of her female offspring. HEMOPHILIA
Bleeding disorder
Bleeding disorder
HEMOPHILIA The bleeding trait in hemophilia can have various degrees of severity, depending on the character of the genetic deficiency. Bleeding usually does not occur except after trauma, but in some patients, the degree of trauma required to cause severe and prolonged bleeding may be so mild that it is hardly noticeable. For instance, bleeding can often last for days after extraction of a tooth.
Factor VIII has 2 active components - A large component with a molecular weight in the millions and A smaller component with a molecular weight of about 230,000. The smaller component is most important in the intrinsic pathway for clotting, and it is deficiency of this part of Factor VIII that causes classic hemophilia. Another bleeding disease with somewhat different characteristics, called von Willebrands disease, results from loss of the large component. HEMOPHILIA
Hemarthrosis of the joints
Bleeding disorder
Classic hemophilia experiences severe prolonged bleeding, almost the only therapy that is truly effective is injection of purified Factor VIII. The cost of Factor VIII is high, because it is gathered from human blood and only in extremely small quantities. Use of Recombinant Factor VIII will make this treatment available to more patients with classic hemophilia. TREATMENT
Thrombocytopenia means the presence of very low no. of platelets in the circulating blood. People with thrombocytopenia have a tendency to bleed, except that the bleeding is usually from many small venules or capillaries. As a result, small punctate hemorrhages occur throughout the body tissues. The skin of such a person displays many small, purplish blotches, giving the disease the name thrombocytopenic purpura. THROMBOCYTOPENIA
Bleeding will not occur until the number of platelets in the blood falls below 50,000/亮l, rather than the normal 150,000 to 300,000. Levels as low as 10,000/亮l are frequently lethal. Idiopathic thrombocytopenia, which means thrombocytopenia of unknown cause. Specific antibodies have formed and react against the platelets themselves to destroy them. TREATMENT-By giving fresh whole blood transfusions that contain large numbers of platelets. Splenectomy is often helpful, almost complete cure. THROMBOCYTOPENIA
An abnormal clot that develops in a blood vessel is called a thrombus. Once a clot has developed, continued flow of blood past the clot is likely to break it away from its attachment and cause the clot to flow with the blood; such freely flowing clots are known as emboli. emboli that originate in large arteries or in the left side of the heart can flow peripherally and plug arteries or arterioles in the brain, kidneys, or elsewhere. Emboli that originate in the venous system or in the right side of the heart generally flow into the lungs to cause pulmonary arterial embolism. Thromboembolic conditions
The causes of thromboembolic conditions in the human being are usually two: (1) Any roughened endothelial surface of a vessel as may be caused by arteriosclerosis, infection, or trauma. (2) Blood often clots when it flows very slowly through blood vessels, where small quantities of thrombin and other procoagulants are always being formed. Causes
Use of t-PA in Treating Intravascular Clots. Genetically engineered t-PA (tissue plasminogen activator) is available. It is effective in activating plasminogen to plasmin, which in turn can dissolve some intra- vascular clots. TREATMENT
Bleeding disorder
Occasionally the clotting mechanism becomes activated in widespread areas of the circulation, giving rise to the condition called disseminated intravascular coagulation. This often results from the presence of large amounts of traumatized or dying tissue in the body that releases great quantities of tissue factor into the blood. Frequently, the clots are small but numerous, and they plug a large share of the small peripheral blood vessels. This occurs especially in patients with widespread septicemia, in which either circulating bacteria or bacterial toxinsespecially endotoxinsactivate the clotting mechanisms. Disseminated Intravascular Coagulation
Bleeding disorder
Plugging of small peripheral vessels greatly diminishes delivery of oxygen and other nutrients to the tissuesa situation that leads to or exacerbates circulatory shock. It is partly for this reason that septicemic shock is lethal in 85 percent or more of patients. A peculiar effect of DIC is that the patient on occasion begins to bleed. The reason for this is that so many of the clotting factors are removed by the widespread clotting that too few procoagulants remain to allow normal hemostasis of the remaining blood. Disseminated Intravascular Coagulation
Bleeding disorder

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Bleeding disorder

  • 2. Excessive bleeding can result from deficiency of any one of the many blood-clotting factors. Three particular types of bleeding tendencies that have been studied to the greatest extent are discussed here: bleeding caused by (1) Vitamin K deficiency, (2) Hemophilia, and (3) Thrombocytopenia (platelet deficiency). Conditions which causes Excessive bleeding in Humans
  • 3. Decreased Prothrombin, Factor VII, Factor IX, and Factor X Caused by Vitamin K Deficiency. With few exceptions, almost all the blood-clotting factors are formed by the liver. Therefore, diseases of the liver such as Hepatitis, Cirrhosis, and Acute yellow atrophy Depress the clotting system so greatly that the patient develops a severe tendency to bleed. Vitamin K Deficiency
  • 4. Another cause of depressed formation of clotting factors by the liver is vitamin K deficiency. Vitamin K is an essential factor to a liver carboxylase that adds a carboxyl group to glutamic acid residues on five of the important clotting factors: Prothrombin, Factor VII, Factor IX, Factor X, and Protein C. Vitamin K Deficiency
  • 5. In adding the carboxyl group to glutamic acid residues on the immature clotting factors, vitamin K is oxidized and becomes inactive. Another enzyme, vitamin K epoxide reductase complex 1 (VKOR c1), reduces vitamin K back to its active form. In the absence of active vitamin K, subsequent insuf- ficiency of these coagulation factors in the blood can lead to serious bleeding tendencies. Vitamin K Deficiency
  • 8. Hemophilia is a bleeding disease that occurs almost exclusively in males. In 85% of cases, it is caused by an abnormality or deficiency of Factor VIII; this type of hemophilia is called hemophilia A or classic hemophilia. In the other 15% of hemophilia patients, the bleeding tendency is caused by deficiency of Factor IX. Both of these factors are transmitted genetically by way of the female chromosome. HEMOPHILIA
  • 9. Females will be a hemophilia carrier, transmitting the disease to half of her male offspring and transmitting the carrier state to half of her female offspring. HEMOPHILIA
  • 12. HEMOPHILIA The bleeding trait in hemophilia can have various degrees of severity, depending on the character of the genetic deficiency. Bleeding usually does not occur except after trauma, but in some patients, the degree of trauma required to cause severe and prolonged bleeding may be so mild that it is hardly noticeable. For instance, bleeding can often last for days after extraction of a tooth.
  • 13. Factor VIII has 2 active components - A large component with a molecular weight in the millions and A smaller component with a molecular weight of about 230,000. The smaller component is most important in the intrinsic pathway for clotting, and it is deficiency of this part of Factor VIII that causes classic hemophilia. Another bleeding disease with somewhat different characteristics, called von Willebrands disease, results from loss of the large component. HEMOPHILIA
  • 16. Classic hemophilia experiences severe prolonged bleeding, almost the only therapy that is truly effective is injection of purified Factor VIII. The cost of Factor VIII is high, because it is gathered from human blood and only in extremely small quantities. Use of Recombinant Factor VIII will make this treatment available to more patients with classic hemophilia. TREATMENT
  • 17. Thrombocytopenia means the presence of very low no. of platelets in the circulating blood. People with thrombocytopenia have a tendency to bleed, except that the bleeding is usually from many small venules or capillaries. As a result, small punctate hemorrhages occur throughout the body tissues. The skin of such a person displays many small, purplish blotches, giving the disease the name thrombocytopenic purpura. THROMBOCYTOPENIA
  • 18. Bleeding will not occur until the number of platelets in the blood falls below 50,000/亮l, rather than the normal 150,000 to 300,000. Levels as low as 10,000/亮l are frequently lethal. Idiopathic thrombocytopenia, which means thrombocytopenia of unknown cause. Specific antibodies have formed and react against the platelets themselves to destroy them. TREATMENT-By giving fresh whole blood transfusions that contain large numbers of platelets. Splenectomy is often helpful, almost complete cure. THROMBOCYTOPENIA
  • 19. An abnormal clot that develops in a blood vessel is called a thrombus. Once a clot has developed, continued flow of blood past the clot is likely to break it away from its attachment and cause the clot to flow with the blood; such freely flowing clots are known as emboli. emboli that originate in large arteries or in the left side of the heart can flow peripherally and plug arteries or arterioles in the brain, kidneys, or elsewhere. Emboli that originate in the venous system or in the right side of the heart generally flow into the lungs to cause pulmonary arterial embolism. Thromboembolic conditions
  • 20. The causes of thromboembolic conditions in the human being are usually two: (1) Any roughened endothelial surface of a vessel as may be caused by arteriosclerosis, infection, or trauma. (2) Blood often clots when it flows very slowly through blood vessels, where small quantities of thrombin and other procoagulants are always being formed. Causes
  • 21. Use of t-PA in Treating Intravascular Clots. Genetically engineered t-PA (tissue plasminogen activator) is available. It is effective in activating plasminogen to plasmin, which in turn can dissolve some intra- vascular clots. TREATMENT
  • 23. Occasionally the clotting mechanism becomes activated in widespread areas of the circulation, giving rise to the condition called disseminated intravascular coagulation. This often results from the presence of large amounts of traumatized or dying tissue in the body that releases great quantities of tissue factor into the blood. Frequently, the clots are small but numerous, and they plug a large share of the small peripheral blood vessels. This occurs especially in patients with widespread septicemia, in which either circulating bacteria or bacterial toxinsespecially endotoxinsactivate the clotting mechanisms. Disseminated Intravascular Coagulation
  • 25. Plugging of small peripheral vessels greatly diminishes delivery of oxygen and other nutrients to the tissuesa situation that leads to or exacerbates circulatory shock. It is partly for this reason that septicemic shock is lethal in 85 percent or more of patients. A peculiar effect of DIC is that the patient on occasion begins to bleed. The reason for this is that so many of the clotting factors are removed by the widespread clotting that too few procoagulants remain to allow normal hemostasis of the remaining blood. Disseminated Intravascular Coagulation