Vera Quiz: Polycythemia Vera and Secondary Polycythemia Test

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Questions and Answers

What is the role of endothelium in preventing spontaneous clot formation in normal vessels?

It activates the coagulation cascade

It inhibits the formation of prothrombin activator

It prevents the formation of spontaneous clots (correct)

It expresses serum, which prevents clot formation

In the clotting cascade, what is the main function of fibrinogen?

To express serum and assist in clot retraction

To change prothrombin to thrombin

To activate prothrombin factor

To change fibrinogen to fibrin (correct)

Which pathway is most dominant in the clotting cascade and is activated by tissue factor released by damaged endothelial cells?

Vasoconstriction pathway

Intrinsic pathway

Extrinsic pathway (correct)

Prothrombin pathway

What is the role of thrombin in the clotting cascade?

To change fibrinogen to fibrin Signup and view all the answers

Which chemical mediator is associated with clotting and induces the formation of prothrombin activator?

Tissue factor Signup and view all the answers

What role does thromboxane play in the clotting process?

It activates other platelets to become sticky Signup and view all the answers

How do glycoproteins on the surface of platelets contribute to hemostasis?

They repulse adherence to 'normal' endothelium Signup and view all the answers

What is the function of factor IV in the clotting process?

To activate clotting factors Signup and view all the answers

How do calcium and platelet function relate in the clotting process?

Calcium is required to activate clotting factors Signup and view all the answers

What is the lifespan of platelets in the blood?

8-10 days Signup and view all the answers

What is the main cause of the increased protease activity in the blood in disseminated intravascular coagulation (DIC)?

Unregulated release of thrombin with subsequent fibrin formation Signup and view all the answers

Which laboratory test is typically elevated in disseminated intravascular coagulation (DIC) as a result of increased fibrinolysis?

D-dimer Signup and view all the answers

What is the consequence of widespread thromboses in disseminated intravascular coagulation (DIC)?

Ischemia, infarction, and organ hypoperfusion Signup and view all the answers

What is the primary underlying cause of sickle cell anemia?

Homozygosity for a specific genetic mutation Signup and view all the answers

What is the characteristic feature observed on blood smear in sickle cell anemia?

Howell-Jolly bodies Signup and view all the answers

Which enzyme is responsible for changing fibrinogen to fibrin?

Thrombin Signup and view all the answers

In thrombotic thrombocytopenic purpura (TTP), what is the genetic mutation associated with the disease?

ADAMTS13 gene mutation Signup and view all the answers

What is the mechanism of action of plasmapheresis in TTP?

Replacement of deficient ADAMTS13 enzyme Signup and view all the answers

In essential thrombocythemia (ET), which cells are produced in excess in the bone marrow?

Megakaryocytes Signup and view all the answers

What is the main mechanism of immune thrombocytopenic purpura (ITP)?

Autoimmune attack on platelets Signup and view all the answers

What is the role of Von Willebrand factor in the clotting process?

Activates platelet plug formation and acts as an anchor at sites of vascular injury Signup and view all the answers

What is the consequence of Von Willebrand disease?

Decrease in ability for platelets to bind to vessel wall and missing or defective vWF Signup and view all the answers

What is the main function of Protein C in the clotting process?

Degrades activated clotting factors and prevents excessive clotting Signup and view all the answers

What is the consequence of Protein C deficiency?

Increased risk of venous thrombosis Signup and view all the answers

What is the effect of Factor V Leiden mutation?

Unable to be inactivated by protein C Signup and view all the answers

What is the consequence of acquiring a mutation of JAK2 in polycythemia vera?

Excessive clotting in veins (DVTs) Signup and view all the answers

In secondary polycythemia resulting from high altitude living, what is the compensatory response to hypoxia?

Compensatory increase in red blood cells Signup and view all the answers

What is the consequence of tumor presence as a source of elevated EPO?

Increased red blood cell production Signup and view all the answers

Which condition is more likely to develop gouty arthritis due to an associated characteristic?

Polycythemia vera due to elevated EPO receptor activity Signup and view all the answers

In the context of RBC disorders, what is the consequence of RBC inappropriately high with low EPO level?

Excessive clotting in arteries Signup and view all the answers

What is the mechanism behind the development of immune-mediated hemolytic anemia?

Accelerated destruction of RBCs Signup and view all the answers

In G6PD deficiency, the lack of NADPH leads to a loss of cells' antioxidant protection, resulting in:

Shortened life span of RBC and episodic hemolytic anemia with oxidative stresses Signup and view all the answers

What triggers the formation of Heinz bodies in hemolytic anemia?

Fava beans, infection, drugs Signup and view all the answers

What is the characteristic feature of autoimmune hemolytic anemias?

RBC bursts and warm reactive type Signup and view all the answers

What distinguishes the bone marrow in anemia of chronic disease from that in iron deficiency anemia?

Decreased erythropoiesis and altered iron metabolism Signup and view all the answers

What is the main consequence of the lack of NADPH in G6PD deficiency?

Shortened life span of RBC and episodic hemolytic anemia with oxidative stresses Signup and view all the answers

What is the hallmark of sickle cell anemia?

RBC bursts Signup and view all the answers

What is the role of hepcidin in the pathogenesis of anemia of chronic disease?

Regulates entry of iron into circulation, leading to iron trapping in macrophages and liver cells Signup and view all the answers

Which triggers may lead to hemolytic anemia in G6PD deficiency?

Fava beans, infection, drugs Signup and view all the answers

What can distinguish between anemia of chronic disease and iron deficiency anemia when TIBC is not a good indicator?

Elevated ferritin levels and decreased gut absorption of iron Signup and view all the answers

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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Test your knowledge about Polycythemia Vera and Secondary Polycythemia, including symptoms, causes, and diagnostic criteria. Identify key characteristics and differences between the two conditions.

Polycythemia Vera and Secondary Polycythemia Quiz

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