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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?
- Factor VIIa
- Thrombin
- Factor Xa
- Factor IXa (correct)
What is the source of prothrombin fragment F1.2?
What is the source of prothrombin fragment F1.2?
- Factor X
- Thrombin (correct)
- Fibrinogen
- Factor VIIa
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?
- Impaired membrane activation
- Decreased numbers of alpha granules
- Decreased numbers of dense granules
- Increased levels of thromboxane A2 (correct)
What protein, secreted by endothelial cells, activates fibrinolysis?
What protein, secreted by endothelial cells, activates fibrinolysis?
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?
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?
What is the underlying cause of platelet dysfunction in uremia?
What is the underlying cause of platelet dysfunction in uremia?
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?
Which of the following is a defect in GP IIb/IIIa?
Which of the following is a defect in GP IIb/IIIa?
Which of these is the most common hereditary platelet function defect?
Which of these is the most common hereditary platelet function defect?
Which intimal cell synthesizes and stores von Willebrand factor (VWF)?
Which intimal cell synthesizes and stores von Willebrand factor (VWF)?
Which subendothelial structural protein triggers coagulation through activation of factor VII?
Which subendothelial structural protein triggers coagulation through activation of factor VII?
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?
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?
What is the primary role of Vitamin K in the coagulation process?
What is the primary role of Vitamin K in the coagulation process?
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:
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?
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?
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?
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?
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?
TAR refers to which of the following conditions?
TAR refers to which of the following conditions?
Neonatal autoimmune thrombocytopenia occurs when:
Neonatal autoimmune thrombocytopenia occurs when:
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?
Which coagulation factor is activated by thrombin and stabilizes the fibrin clot?
Which coagulation factor is activated by thrombin and stabilizes the fibrin clot?
Which mechanism of antiplatelet drugs directly targets GP IIb/IIIa?
Which mechanism of antiplatelet drugs directly targets GP IIb/IIIa?
What is the hallmark of Idiopathic Thrombocytopenic Purpura (ITP)?
What is the hallmark of Idiopathic Thrombocytopenic Purpura (ITP)?
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?
Which congenital qualitative platelet disorder is associated with decreased platelet production?
Which congenital qualitative platelet disorder is associated with decreased platelet production?
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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