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Questions and Answers
Which of these is a serine protease that forms a complex with factor VIIIa?
Which of these is a serine protease that forms a complex with factor VIIIa?
What is the source of prothrombin fragment F1.2?
What is the source of prothrombin fragment F1.2?
Which of the following is not a cause of impaired platelet function in myeloproliferative neoplasms?
Which of the following is not a cause of impaired platelet function in myeloproliferative neoplasms?
What protein, secreted by endothelial cells, activates fibrinolysis?
What protein, secreted by endothelial cells, activates fibrinolysis?
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What is the substrate of the complex formed by factor IXa and factor VIIIa?
What is the substrate of the complex formed by factor IXa and factor VIIIa?
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What two regulatory proteins form a complex that digests activated factors V and VIII?
What two regulatory proteins form a complex that digests activated factors V and VIII?
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What is the underlying cause of platelet dysfunction in uremia?
What is the underlying cause of platelet dysfunction in uremia?
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Which of the following is NOT a common cause of thrombocytopenia associated with cardiopulmonary bypass?
Which of the following is NOT a common cause of thrombocytopenia associated with cardiopulmonary bypass?
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Which of the following is a defect in GP IIb/IIIa?
Which of the following is a defect in GP IIb/IIIa?
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Which of these is the most common hereditary platelet function defect?
Which of these is the most common hereditary platelet function defect?
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Which intimal cell synthesizes and stores von Willebrand factor (VWF)?
Which intimal cell synthesizes and stores von Willebrand factor (VWF)?
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Which subendothelial structural protein triggers coagulation through activation of factor VII?
Which subendothelial structural protein triggers coagulation through activation of factor VII?
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What coagulation plasma protein should be assayed when platelets fail to aggregate properly?
What coagulation plasma protein should be assayed when platelets fail to aggregate properly?
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What laboratory test findings would you expect to see in a patient with Bernard-Soulier Syndrome?
What laboratory test findings would you expect to see in a patient with Bernard-Soulier Syndrome?
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What is the primary role of Vitamin K in the coagulation process?
What is the primary role of Vitamin K in the coagulation process?
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The reduction in thrombin generation observed in patients with Scott syndrome results from:
The reduction in thrombin generation observed in patients with Scott syndrome results from:
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What is the likely diagnosis for a 2-year-old child presenting with an unexpected platelet count of 15,000/uL and a recent history of a viral infection?
What is the likely diagnosis for a 2-year-old child presenting with an unexpected platelet count of 15,000/uL and a recent history of a viral infection?
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Which of the following is NOT a typical clinical presentation of platelet-related bleeding?
Which of the following is NOT a typical clinical presentation of platelet-related bleeding?
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Which of the following drugs causes a reduction in platelet count by inhibiting megakaryocyte maturation?
Which of the following drugs causes a reduction in platelet count by inhibiting megakaryocyte maturation?
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A defect in primary hemostasis (platelet response to an injury) most commonly results in which of the following?
A defect in primary hemostasis (platelet response to an injury) most commonly results in which of the following?
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When a drug acts as a hapten to induce thrombocytopenia, an antibody forms against which of the following?
When a drug acts as a hapten to induce thrombocytopenia, an antibody forms against which of the following?
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TAR refers to which of the following conditions?
TAR refers to which of the following conditions?
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Neonatal autoimmune thrombocytopenia occurs when:
Neonatal autoimmune thrombocytopenia occurs when:
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Treatment with an anticomplement agent such as eculizumab is first-line therapy for which of the following?
Treatment with an anticomplement agent such as eculizumab is first-line therapy for which of the following?
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Which coagulation factor is activated by thrombin and stabilizes the fibrin clot?
Which coagulation factor is activated by thrombin and stabilizes the fibrin clot?
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Which mechanism of antiplatelet drugs directly targets GP IIb/IIIa?
Which mechanism of antiplatelet drugs directly targets GP IIb/IIIa?
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What is the hallmark of Idiopathic Thrombocytopenic Purpura (ITP)?
What is the hallmark of Idiopathic Thrombocytopenic Purpura (ITP)?
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Which of the following endogenous plasma inhibitors is important for controlling excessive thrombin generation?
Which of the following endogenous plasma inhibitors is important for controlling excessive thrombin generation?
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Which congenital qualitative platelet disorder is associated with decreased platelet production?
Which congenital qualitative platelet disorder is associated with decreased platelet production?
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Flashcards
Glanzmann thrombasthenia
Glanzmann thrombasthenia
A bleeding disorder caused by a defect in GP IIb/IIIa, leading to impaired platelet aggregation.
von Willebrand factor (VWF)
von Willebrand factor (VWF)
A blood glycoprotein essential for platelet adhesion and aggregation.
Ristocetin test
Ristocetin test
A laboratory test used to evaluate platelet function in regards to VWF.
Bernard-Soulier syndrome
Bernard-Soulier syndrome
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Thrombin generation
Thrombin generation
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Vitamin K's role
Vitamin K's role
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Factor VII activation
Factor VII activation
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Storage pool deficiency
Storage pool deficiency
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Antiplatelet drugs mechanism
Antiplatelet drugs mechanism
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Coagulation factors synthesis
Coagulation factors synthesis
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Thrombin and Factor XIII
Thrombin and Factor XIII
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May-Hegglin anomaly
May-Hegglin anomaly
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Endogenous plasma inhibitors
Endogenous plasma inhibitors
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Calcium Binding
Calcium Binding
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Prothrombin Fragment F1.2
Prothrombin Fragment F1.2
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Factor IXa and VIIIa Complex
Factor IXa and VIIIa Complex
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TPA (Tissue Plasminogen Activator)
TPA (Tissue Plasminogen Activator)
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Anti-GP IIb/IIIa Antibodies
Anti-GP IIb/IIIa Antibodies
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VWF (Von Willebrand Factor)
VWF (Von Willebrand Factor)
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Thromboxane A2
Thromboxane A2
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Impaired Platelet Activation
Impaired Platelet Activation
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HPA-1a
HPA-1a
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Acute ITP
Acute ITP
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Thrombocytopenia
Thrombocytopenia
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Anagrelide
Anagrelide
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Neonatal autoimmune thrombocytopenia
Neonatal autoimmune thrombocytopenia
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HUS in children
HUS in children
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Eculizumab
Eculizumab
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Thrombocytosis
Thrombocytosis
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Study Notes
Hematology Review Questions: Chapters 35, 37, 38
-
Chapter 35: Focuses on platelet disorders.
- Glanzmann thrombasthenia: A defect in GP IIb/IIIa causing abnormal platelet function.
- Bernard-Soulier syndrome: Patients have defects in GP Ib/IX/V, leading to abnormal platelet function, characterized by large platelets.
- Storage pool defects: A common hereditary platelet function defect; characterized by reduced numbers of dense granules & alpha granules
- Alterations in platelet membrane phospholipid expression: Can impair platelet activation and aggregation.
- Platelet function defects in uremia: Increased paraprotein levels and impaired membrane activation owing to protein coating are associated.
- Impaired thrombin generation in Scott syndrome: Caused by decreased numbers of alpha granules and dense granules.
- Thrombocytopenia after cardiopulmonary bypass is not related to decreased platelet production or increased clearance by immune complexes
- Anti-GP IIb/IIIa antibodies: Can cause platelet aggregation dysfunction.
- Aspirin blocks thromboxane A2 synthesis.
- Congenital qualitative platelet disorder is Ehlers-Danlos syndrome.
- May-Hegglin anomaly: Autosomal dominant disorder linked to platelet production defects.
- Large, overactive platelets: May be related to large platelets.
- ITP (Immune thrombocytopenic purpura): Not a hallmark; it's characterized by decreased platelet production.
- NAIT (Neonatal Alloimmune Thrombocytopenia): The most common antigen causing NAIT.
- Acute ITP: A child with 15,000/uL platelets recently having a viral infection.
-
Chapter 37: Discusses platelet-related bleeding.
- Bleeding presentations: Mucosal bleeding often results from defects in primary hemostasis.
- Anagrelide: Inhibits megakaryocyte maturation, leading to a reduction in platelet count.
- Neonatal autoimmune thrombocytopenia (NAT): Results from maternal antibodies crossing the placenta and affecting the infant's platelets.
- Thrombocytopenia/thrombocytosis: Associated with autoimmune reactions and other disorders.
-
Chapter 38: Focuses on specific causes of platelet disorders.
- Anagrelide: Inhibits megakaryocyte maturation and reduces platelet count.
- Mucosal bleeding: Often results from primary hemostasis defects.
- Drug-induced thrombocytopenia: Antibody formation to a drug-protein complex may cause immune-mediated thrombocytopenia.
- HUS (Hemolytic uremic syndrome): In children, associated with treatment requiring anticomplement agents.
- Atypical HUS: Associated with treatment requiring anticomplement agents..
- Atypical Hemolytic Uremic Syndrome (aHUS): This is a condition that can be treated with anticomplement agents.
- Thrombocytosis: Characteristics of thrombocytosis, including self-correcting properties.
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Description
Test your knowledge on platelet disorders from Chapters 35, 37, and 38 of the Hematology review. Explore conditions like Glanzmann thrombasthenia, Bernard-Soulier syndrome, and various storage pool defects. Understand the implications of these disorders on platelet function and thrombus formation.