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Questions and Answers
What is the underlying abnormality in vascular disorders?
What is the characteristic of thrombasthenia?
What is the cause of acquired thrombasthenia?
What is a cause of thrombocytopenia due to decreased platelet production?
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What is a cause of immune thrombocytopenia?
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What is a symptoms of vascular disorders?
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What is the primary consideration for treatment decisions in ITP patients?
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What is the recommended treatment approach for asymptomatic ITP patients with a platelet count above 30 × 109/L?
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Which of the following is a therapeutic option for ITP patients in emergency settings?
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What is a potential side effect of long-term steroid use in ITP patients?
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Which of the following medications is a Thrombopoietin receptor agonist (TPO-RA)?
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What is a potential surgical treatment option for ITP patients?
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What is the primary cause of thrombocytopenia in immune thrombocytopenic purpura?
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What is the most common initial manifestation of immune thrombocytopenic purpura?
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Which of the following is a secondary cause of immune thrombocytopenic purpura?
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What is the goal of immune thrombocytopenic purpura management in adults?
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What is the risk of fatal bleeding in immune thrombocytopenic purpura?
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What is a diagnostic test for immune thrombocytopenic purpura?
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What is a condition that may be associated with thrombocytopenia?
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What is the definition of primary immune thrombocytopenic purpura?
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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.
