Questions and Answers
What is the role of endothelium in preventing spontaneous clot formation in normal vessels?
It prevents the formation of spontaneous clots
In the clotting cascade, what is the main function of fibrinogen?
To change fibrinogen to fibrin
Which pathway is most dominant in the clotting cascade and is activated by tissue factor released by damaged endothelial cells?
Extrinsic pathway
What is the role of thrombin in the clotting cascade?
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Which chemical mediator is associated with clotting and induces the formation of prothrombin activator?
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What role does thromboxane play in the clotting process?
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How do glycoproteins on the surface of platelets contribute to hemostasis?
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What is the function of factor IV in the clotting process?
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How do calcium and platelet function relate in the clotting process?
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What is the lifespan of platelets in the blood?
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What is the main cause of the increased protease activity in the blood in disseminated intravascular coagulation (DIC)?
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Which laboratory test is typically elevated in disseminated intravascular coagulation (DIC) as a result of increased fibrinolysis?
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What is the consequence of widespread thromboses in disseminated intravascular coagulation (DIC)?
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What is the primary underlying cause of sickle cell anemia?
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What is the characteristic feature observed on blood smear in sickle cell anemia?
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Which enzyme is responsible for changing fibrinogen to fibrin?
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In thrombotic thrombocytopenic purpura (TTP), what is the genetic mutation associated with the disease?
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What is the mechanism of action of plasmapheresis in TTP?
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In essential thrombocythemia (ET), which cells are produced in excess in the bone marrow?
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What is the main mechanism of immune thrombocytopenic purpura (ITP)?
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What is the role of Von Willebrand factor in the clotting process?
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What is the consequence of Von Willebrand disease?
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What is the main function of Protein C in the clotting process?
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What is the consequence of Protein C deficiency?
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What is the effect of Factor V Leiden mutation?
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What is the consequence of acquiring a mutation of JAK2 in polycythemia vera?
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In secondary polycythemia resulting from high altitude living, what is the compensatory response to hypoxia?
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What is the consequence of tumor presence as a source of elevated EPO?
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Which condition is more likely to develop gouty arthritis due to an associated characteristic?
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In the context of RBC disorders, what is the consequence of RBC inappropriately high with low EPO level?
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What is the mechanism behind the development of immune-mediated hemolytic anemia?
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In G6PD deficiency, the lack of NADPH leads to a loss of cells' antioxidant protection, resulting in:
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What triggers the formation of Heinz bodies in hemolytic anemia?
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What is the characteristic feature of autoimmune hemolytic anemias?
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What distinguishes the bone marrow in anemia of chronic disease from that in iron deficiency anemia?
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What is the main consequence of the lack of NADPH in G6PD deficiency?
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What is the hallmark of sickle cell anemia?
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What is the role of hepcidin in the pathogenesis of anemia of chronic disease?
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Which triggers may lead to hemolytic anemia in G6PD deficiency?
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What can distinguish between anemia of chronic disease and iron deficiency anemia when TIBC is not a good indicator?
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Study Notes
Endothelium and Clotting Cascade
- Endothelium prevents spontaneous clot formation in normal vessels by inhibiting platelet activation and clotting factor expression.
- Fibrinogen is converted to fibrin, forming a clot, in the clotting cascade.
- The extrinsic pathway, activated by tissue factor released by damaged endothelial cells, is the most dominant pathway in the clotting cascade.
- Thrombin converts fibrinogen to fibrin, activates platelets, and stimulates the release of procoagulant factors.
Platelets and Clotting
- Thromboxane induces platelet aggregation and vasoconstriction, contributing to the clotting process.
- Glycoproteins on the surface of platelets facilitate platelet aggregation and adhesion to the site of injury.
- Factor IV (calcium) is essential for the clotting process, as it enables the interaction of clotting factors.
- The lifespan of platelets in the blood is approximately 7-10 days.
Disseminated Intravascular Coagulation (DIC)
- Disseminated intravascular coagulation (DIC) is characterized by increased protease activity in the blood due to excessive thrombin production.
- Elevated D-dimer levels are a hallmark of DIC, indicating increased fibrinolysis.
- Widespread thromboses in DIC can lead to multiple organ failure.
Sickle Cell Anemia
- Sickle cell anemia is caused by a mutation in the HBB gene, leading to abnormal hemoglobin production.
- The characteristic feature of sickle cell anemia is the presence of sickle-shaped red blood cells on a blood smear.
- Thrombin converts fibrinogen to fibrin, which is essential for clot formation.
Thrombotic Thrombocytopenic Purpura (TTP) and Essential Thrombocythemia (ET)
- The genetic mutation associated with TTP is an ADAMTS13 deficiency.
- Plasmapheresis is used to treat TTP by removing antibodies and replacing ADAMTS13.
- In ET, the bone marrow produces excessive platelets, leading to thrombotic complications.
Immune Thrombocytopenic Purpura (ITP) and Von Willebrand Disease
- ITP is characterized by immune-mediated platelet destruction, leading to thrombocytopenia.
- Von Willebrand factor facilitates platelet adhesion and aggregation, playing a crucial role in the clotting process.
- Von Willebrand disease is characterized by bleeding disorders due to deficient or dysfunctional Von Willebrand factor.
Protein C and Factor V Leiden Mutation
- Protein C is a natural anticoagulant that degrades activated clotting factors, preventing thrombosis.
- Deficiency in Protein C leads to increased thrombotic risk.
- The Factor V Leiden mutation increases the risk of thrombosis by making it resistant to Protein C-mediated inactivation.
Polycythemia Vera and Secondary Polycythemia
- The JAK2 mutation is associated with polycythemia vera, leading to excessive red blood cell production.
- In secondary polycythemia, high altitude living triggers an increase in red blood cell production to compensate for hypoxia.
Red Blood Cell Disorders
- G6PD deficiency leads to a lack of NADPH, resulting in reduced antioxidant defenses and hemolytic anemia.
- Heinz bodies form in hemolytic anemia due to the accumulation of denatured hemoglobin.
- Autoimmune hemolytic anemia is characterized by the presence of autoantibodies against red blood cells.
- Hepcidin regulates iron metabolism and is involved in the pathogenesis of anemia of chronic disease.
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