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