Hematology Review: Platelet Disorders
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Questions and Answers

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?

  • Factor X
  • Thrombin (correct)
  • Fibrinogen
  • Factor VIIa
  • 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?

    <p>Tissue plasminogen activator (TPA) (B)</p> Signup and view all the answers

    What is the substrate of the complex formed by factor IXa and factor VIIIa?

    <p>Factor X (C)</p> Signup and view all the answers

    What two regulatory proteins form a complex that digests activated factors V and VIII?

    <p>Protein C and Protein S (C)</p> Signup and view all the answers

    What is the underlying cause of platelet dysfunction in uremia?

    <p>Higher than normal levels of nitric oxide (NO) (C)</p> Signup and view all the answers

    Which of the following is NOT a common cause of thrombocytopenia associated with cardiopulmonary bypass?

    <p>Aspirin ingestion (A)</p> Signup and view all the answers

    Which of the following is a defect in GP IIb/IIIa?

    <p>Glanzmann thrombasthenia (D)</p> Signup and view all the answers

    Which of these is the most common hereditary platelet function defect?

    <p>Storage pool disease (B)</p> Signup and view all the answers

    Which intimal cell synthesizes and stores von Willebrand factor (VWF)?

    <p>Endothelial cell (D)</p> Signup and view all the answers

    Which subendothelial structural protein triggers coagulation through activation of factor VII?

    <p>Tissue factor (C)</p> Signup and view all the answers

    What coagulation plasma protein should be assayed when platelets fail to aggregate properly?

    <p>Fibrinogen (A)</p> Signup and view all the answers

    What laboratory test findings would you expect to see in a patient with Bernard-Soulier Syndrome?

    <p>Abnormal platelet response to ristocetin (D)</p> Signup and view all the answers

    What is the primary role of Vitamin K in the coagulation process?

    <p>To carboxylate the factors to allow for the prothrombin group factors (A)</p> Signup and view all the answers

    The reduction in thrombin generation observed in patients with Scott syndrome results from:

    <p>Altered expression of phospholipids on the platelet membrane (D)</p> Signup and view all the answers

    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?

    <p>Acute ITP (A)</p> Signup and view all the answers

    Which of the following is NOT a typical clinical presentation of platelet-related bleeding?

    <p>Abnormal bone formation, including hypoplasia of the forearms (C)</p> Signup and view all the answers

    Which of the following drugs causes a reduction in platelet count by inhibiting megakaryocyte maturation?

    <p>Anagrelide (B)</p> Signup and view all the answers

    A defect in primary hemostasis (platelet response to an injury) most commonly results in which of the following?

    <p>Mucosal bleeding (D)</p> Signup and view all the answers

    When a drug acts as a hapten to induce thrombocytopenia, an antibody forms against which of the following?

    <p>The platelet membrane protein to which it is bound (C)</p> Signup and view all the answers

    TAR refers to which of the following conditions?

    <p>Thrombocytopenia-absent radius syndrome (C)</p> Signup and view all the answers

    Neonatal autoimmune thrombocytopenia occurs when:

    <p>The mother has an autoimmune antibody to her own platelets, which crosses the placenta and reacts with the infant’s platelets (B)</p> Signup and view all the answers

    Treatment with an anticomplement agent such as eculizumab is first-line therapy for which of the following?

    <p>Atypical HUS (B)</p> Signup and view all the answers

    Which coagulation factor is activated by thrombin and stabilizes the fibrin clot?

    <p>Factor XIII (B)</p> Signup and view all the answers

    Which mechanism of antiplatelet drugs directly targets GP IIb/IIIa?

    <p>Direct binding (B)</p> Signup and view all the answers

    What is the hallmark of Idiopathic Thrombocytopenic Purpura (ITP)?

    <p>Autoimmune destruction of platelets (A)</p> Signup and view all the answers

    Which of the following endogenous plasma inhibitors is important for controlling excessive thrombin generation?

    <p>Antithrombin (AT) (C), Tissue Factor Pathway Inhibitor (TFPI) (D)</p> Signup and view all the answers

    Which congenital qualitative platelet disorder is associated with decreased platelet production?

    <p>May-Hegglin anomaly (D)</p> Signup and view all the answers

    Flashcards

    Glanzmann thrombasthenia

    A bleeding disorder caused by a defect in GP IIb/IIIa, leading to impaired platelet aggregation.

    von Willebrand factor (VWF)

    A blood glycoprotein essential for platelet adhesion and aggregation.

    Ristocetin test

    A laboratory test used to evaluate platelet function in regards to VWF.

    Bernard-Soulier syndrome

    A hereditary blood disorder characterized by large platelets and impaired aggregation.

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    Thrombin generation

    The process of producing thrombin, crucial for blood clotting.

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    Vitamin K's role

    Essential for the carboxylation of prothrombin group factors which allows them to function properly.

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    Factor VII activation

    Triggered by tissue factor leading to the beginning of the coagulation pathway.

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    Storage pool deficiency

    A condition where platelets have decreased granules, affecting their function.

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    Antiplatelet drugs mechanism

    Drugs that inhibit platelet aggregation by targeting glycoprotein IIb/IIIa.

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    Coagulation factors synthesis

    Most coagulation factors are synthesized in the liver.

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    Thrombin and Factor XIII

    Thrombin activates Factor XIII, stabilizing the fibrin clot.

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    May-Hegglin anomaly

    An autosomal dominant disorder associated with decreased platelet production.

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    Endogenous plasma inhibitors

    Inhibitors like antithrombin (AT) and tissue factor pathway inhibitor (TFPI) control thrombin generation.

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    Calcium Binding

    Process where calcium ions attach to specific proteins in blood, aiding in coagulation.

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    Prothrombin Fragment F1.2

    A peptide released during prothrombin proteolysis, indicative of thrombin generation.

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    Factor IXa and VIIIa Complex

    A complex that activates Factor X in the coagulation cascade, crucial for thrombin generation.

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    TPA (Tissue Plasminogen Activator)

    A protein that promotes fibrinolysis by converting plasminogen to plasmin.

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    Anti-GP IIb/IIIa Antibodies

    Antibodies that impair platelet aggregation by blocking receptors on platelets.

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    VWF (Von Willebrand Factor)

    A protein that mediates platelet adhesion and stabilizes factor VIII in the blood.

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    Thromboxane A2

    A potent vasoconstrictor and promoter of platelet aggregation, inhibited by aspirin.

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    Impaired Platelet Activation

    Decreased platelet function often due to paraproteins or uremia.

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    HPA-1a

    A protein critical for the development of NAIT (Neonatal alloimmune thrombocytopenia).

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    Acute ITP

    A condition characterized by a sudden decrease in platelet count, often resulting in bleeding complications.

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    Thrombocytopenia

    A condition where there is an abnormally low platelet count, which can lead to bleeding disorders.

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    Anagrelide

    A drug that reduces platelet count by inhibiting the maturation of megakaryocytes.

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    Neonatal autoimmune thrombocytopenia

    A condition where maternal antibodies attack the infant's platelets, leading to low counts.

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    HUS in children

    Hemolytic uremic syndrome characterized by diarrhea caused by certain bacterial infections, like Shigella.

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    Eculizumab

    An anticomplement agent used as first-line therapy in atypical hemolytic uremic syndrome (aHUS).

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    Thrombocytosis

    A condition with an excessively high platelet count that is often self-correcting.

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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.

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