Coagulation Disorders: Inherited and Acquired
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Questions and Answers

What is the mode of inheritance of Factor XII deficiency?

  • Mitochondrial inheritance
  • X-linked recessive
  • Autosomal recessive (correct)
  • Autosomal dominant
  • What is the typical clinical presentation of patients with Factor XI deficiency?

  • Asymptomatic until old age
  • No bleeding tendencies
  • Severe bleeding tendency
  • Mild bleeding tendencies that respond to therapy (correct)
  • What is the laboratory test that is characteristic of Prekallikrein deficiency?

  • Prolonged TT
  • Prolonged APTT (correct)
  • Prolonged RT
  • Prolonged PT
  • What is the ethnic group in which Factor XI deficiency is more common?

    <p>Ashkenazi Jews</p> Signup and view all the answers

    What is the treatment for Factor XI deficiency?

    <p>Fresh whole blood</p> Signup and view all the answers

    What percentage of congenital bleeding disorders is accounted for by Hemophilia A?

    <p>85%</p> Signup and view all the answers

    What is the effect of DDAVP on plasma concentration of VIII:C?

    <p>Increases it three to six folds</p> Signup and view all the answers

    What is the APTT result in Hemophilia A?

    <p>Prolonged</p> Signup and view all the answers

    What is the mode of transmission of Hemophilia A?

    <p>X-linked recessive</p> Signup and view all the answers

    What is the approximate incidence of Hemophilia A in the population?

    <p>5 per 100,000</p> Signup and view all the answers

    Study Notes

    Coagulation Disorders

    • Coagulopathies can be classified into two types: inherited disorders, which affect one clotting factor, and acquired disorders, which affect several clotting factors and hemostatic pathways.

    Inherited Disorders of Coagulation

    Intrinsic Pathway Disorders

    • Factor XII deficiency (Hageman factor deficiency) is an autosomal recessive disorder, where homozygous individuals have no factor XII, and heterozygous individuals have variable levels of factor XII.

    • Clinical findings: no bleeding disorder, but laboratory tests show prolonged APTT.

    • Therapy: none, as it is usually an in vitro phenomenon.

    • Prekallikrein deficiency (Fletcher factor deficiency) is an autosomal recessive disorder.

    • Clinical findings: no bleeding disorder.

    • Laboratory tests: prolonged APTT.

    • HMWK deficiency (Fitzgerald, Flaujeac, Williams factor deficiency) is an autosomal recessive disorder.

    • Clinical findings: no bleeding disorder.

    • Laboratory tests: mildly prolonged APTT.

    • Factor XI deficiency (Hemophilia C, Rosenthal's disease) is an autosomal dominant hemophilia, commonly found in the Ashkenazi Jewish population.

    • Clinical findings: mild bleeding tendencies, usually responding to therapy, and asymptomatic until trauma or surgery.

    • Laboratory tests: prolonged APTT.

    • Therapy: fresh whole blood or fresh plasma.

    • Factor VIII:C deficiency (Hemophilia A, Classic Hemophilia) is a sex-linked or X-linked recessive disorder.

    • Clinical findings: severe, moderate, or mild bleeding diathesis, depending on the level of factor VIII:C activity.

    • Bleeding symptoms include bleeding into soft tissues or joints, hematomas, hemarthroses, epistaxis, hematuria, and GIT and intracranial hemorrhage.

    • Laboratory tests: prolonged APTT, with varying levels of factor VIII:C in daughters of carrier females and daughters of hemophiliacs.

    • Therapy: cryoprecipitate, DDAVP, porcine factor VIII:C concentrates, and prothrombin complex concentrates.

    • Factor IX deficiency (Hemophilia B, Christmas disease) is an autosomal recessive disorder.

    • (No specific information provided in the text)

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    Description

    This quiz covers coagulation disorders, including inherited and acquired disorders, and their effects on the coagulation pathway. Topics include coagulopathies, intrinsic pathway disorders, and factor XII deficiency.

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