Vascular Disorders and Bleeding
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Questions and Answers

What is the underlying abnormality in vascular disorders?

  • Abnormalities in the platelet count
  • Abnormalities in the hemostatic testing
  • Abnormalities in the vessels themselves or the perivascular connective tissues (correct)
  • Abnormalities in the megakaryocytes
  • What is the characteristic of thrombasthenia?

  • A disorder of platelet production
  • A disorder of platelet function (correct)
  • A disorder of increased platelet destruction
  • A disorder of blood vessel structure
  • What is the cause of acquired thrombasthenia?

  • Viral infections such as HIV
  • Drugs such as aspirin (correct)
  • Genetic defect
  • Bone marrow disorders
  • What is a cause of thrombocytopenia due to decreased platelet production?

    <p>Megaloblastic anemia</p> Signup and view all the answers

    What is a cause of immune thrombocytopenia?

    <p>Chronic lymphocytic leukemia</p> Signup and view all the answers

    What is a symptoms of vascular disorders?

    <p>Superficial bleeding</p> Signup and view all the answers

    What is the primary consideration for treatment decisions in ITP patients?

    <p>Need for concomitant antithrombotic therapy or other medications</p> Signup and view all the answers

    What is the recommended treatment approach for asymptomatic ITP patients with a platelet count above 30 × 109/L?

    <p>Observation and follow-up of platelet count</p> Signup and view all the answers

    Which of the following is a therapeutic option for ITP patients in emergency settings?

    <p>Intravenous immunoglobulin (IVIg)</p> Signup and view all the answers

    What is a potential side effect of long-term steroid use in ITP patients?

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

    Which of the following medications is a Thrombopoietin receptor agonist (TPO-RA)?

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

    What is a potential surgical treatment option for ITP patients?

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

    What is the primary cause of thrombocytopenia in immune thrombocytopenic purpura?

    <p>Impaired thrombopoiesis by antiplatelet antibodies</p> Signup and view all the answers

    What is the most common initial manifestation of immune thrombocytopenic purpura?

    <p>Mucocutaneous bleeding</p> Signup and view all the answers

    Which of the following is a secondary cause of immune thrombocytopenic purpura?

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

    What is the goal of immune thrombocytopenic purpura management in adults?

    <p>To maintain a hemostatic platelet count while minimizing the toxicity of therapy</p> Signup and view all the answers

    What is the risk of fatal bleeding in immune thrombocytopenic purpura?

    <p>Greatest in elderly patients with persistent and severe thrombocytopenia</p> Signup and view all the answers

    What is a diagnostic test for immune thrombocytopenic purpura?

    <p>Direct coombs test</p> Signup and view all the answers

    What is a condition that may be associated with thrombocytopenia?

    <p>All of the above</p> Signup and view all the answers

    What is the definition of primary immune thrombocytopenic purpura?

    <p>An autoimmune disorder with isolated thrombocytopenia and no secondary cause</p> Signup and view all the answers

    Study Notes

    Vascular Disorders

    • Characterized by easy bruising and spontaneous bleeding from small vessels due to abnormalities in vessels or perivascular connective tissues.
    • Defects can be acquired (abnormalities in endothelial cell lining) or hereditary (connective tissue supporting vessels).

    Symptoms

    • Superficial bleeding
    • Hemostatic testing is normal

    Platelet Disorders

    Thrombasthenia

    • Hereditary thrombasthenia
    • Acquired thrombasthenia (caused by drugs like Aspirin)

    Thrombocytopenia

    • Causes:
      • Disorders of decreased platelet production (congenital thrombocytopenia, viral infections, megaloblastic anemia, myelosuppressive therapy)
      • Disorders of increased platelet destruction (immune thrombocytopenia, associated with SLE, CLL, or lymphoma, infection, or drugs)
      • Disorders of increased platelet consumption (disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome)
      • Dilutional thrombocytopenia
      • Splenic sequestration (portal hypertension)

    Immune Thrombocytopenia (ITP)

    • Defined by low platelet count secondary to accelerated platelet destruction and impaired thrombopoiesis by antiplatelet antibodies
    • Types:
      • Primary ITP (isolated thrombocytopenia, no secondary cause)
      • Secondary ITP (associated with SLE, CLL, lymphoma, infection, or drugs)

    Diagnosis of ITP

    • Mucocutaneous bleeding, petechiae, purpura, epistaxis, and gum bleeding
    • Risk of fatal bleeding greatest in elderly patients with persistent and severe thrombocytopenia
    • Diagnostic tests: direct coombs test, pregnancy test, serum ferritin, H. Pylori, antinuclear antibodies, and PCR for CMV

    Treatment of ITP

    • Goal: maintain hemostatic platelet count while minimizing toxicity and improving health-related quality of life
    • Treatment individualized to patient, considering other factors like need for antithrombotic therapy, invasive procedures, or surgery
    • Asymptomatic patients with platelet counts > 30 × 109/L require no treatment
    • Symptomatic patients or platelet counts < 30 × 109/L require treatment with:
      • Corticosteroids (initial treatment)
      • Intravenous immunoglobulin (IVIg) (emergency settings)
      • Medical therapies (Rituximab, thrombopoietin receptor agonists, azathioprine, cyclosporine A, danazol, dapsone, mycophenolate mofetil)
      • Surgical treatment (splenectomy)

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    Description

    Learn about vascular disorders, a group of conditions characterized by easy bruising and spontaneous bleeding from small vessels, and their underlying abnormalities.

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