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Questions and Answers
What is the primary mechanism of red blood cell destruction in Autoimmune Hemolytic Anemia?
What is the most common inheritance pattern of Glucose-6-Phosphate Dehydrogenase Deficiency?
Which of the following medications can exacerbate hemolysis in Glucose-6-Phosphate Dehydrogenase Deficiency?
What is the primary diagnostic test for Hereditary Spherocytosis?
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What is the primary treatment for Hereditary Spherocytosis?
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What is the characteristic red blood cell morphology in Hereditary Spherocytosis?
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Which of the following is a common symptom of Autoimmune Hemolytic Anemia?
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What is the primary mechanism of red blood cell destruction in Glucose-6-Phosphate Dehydrogenase Deficiency?
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Which of the following is a common complication of Autoimmune Hemolytic Anemia?
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What is the primary treatment for Cold Autoimmune Hemolytic Anemia?
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Study Notes
Hemolytic Disorders
Autoimmune Hemolytic Anemia (AIHA)
- Definition: Autoantibody-mediated destruction of red blood cells
- Types:
- Warm AIHA: IgG autoantibodies bind to RBCs at body temperature
- Cold AIHA: IgM autoantibodies bind to RBCs at low temperatures
- Causes:
- Primary AIHA (idiopathic)
- Secondary AIHA (associated with autoimmune disorders, infections, or malignancies)
- Symptoms:
- Anemia, fatigue, pale skin, jaundice, dark urine
- Diagnosis:
- Positive direct antiglobulin test (DAT)
- Reticulocytosis
- Spherocytes on peripheral blood smear
- Treatment:
- Corticosteroids
- Immunoglobulin therapy
- Splenectomy
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
- Definition: Inherited disorder affecting RBC enzyme G6PD, leading to hemolytic anemia
- Epidemiology:
- X-linked recessive inheritance
- Most common in Mediterranean, African, and Southeast Asian populations
- Causes:
- Oxidative stress triggers hemolysis
- Certain medications (antimalarials, sulfonamides) can exacerbate hemolysis
- Symptoms:
- Hemolytic anemia, jaundice, dark urine
- Diagnosis:
- Enzyme assay
- Fluorescent spot test
- Treatment:
- Avoidance of triggering medications
- Supportive care (hydration, transfusions)
Hereditary Spherocytosis (HS)
- Definition: Inherited disorder affecting RBC membrane, leading to spherocytosis and hemolytic anemia
- Epidemiology:
- Autosomal dominant inheritance
- Most common in people of European descent
- Causes:
- Mutations in RBC membrane protein genes (e.g., ANK1, SPTA1)
- Symptoms:
- Hemolytic anemia, jaundice, splenomegaly
- Diagnosis:
- Osmotic fragility test
- Eosin-5'-maleimide (EMA) binding test
- Treatment:
- Splenectomy
- Supportive care (transfusions, iron chelation)
Hemolytic Disorders
Autoimmune Hemolytic Anemia (AIHA)
- Autoantibody-mediated destruction of red blood cells leads to anemia
- Two types: Warm AIHA (IgG autoantibodies bind to RBCs at body temperature) and Cold AIHA (IgM autoantibodies bind to RBCs at low temperatures)
- Causes: Primary AIHA (idiopathic) and Secondary AIHA (associated with autoimmune disorders, infections, or malignancies)
- Symptoms: Anemia, fatigue, pale skin, jaundice, and dark urine
- Diagnosis: Positive direct antiglobulin test (DAT), reticulocytosis, and spherocytes on peripheral blood smear
- Treatment: Corticosteroids, immunoglobulin therapy, and splenectomy
Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
- Inherited disorder affecting RBC enzyme G6PD, leading to hemolytic anemia
- X-linked recessive inheritance, most common in Mediterranean, African, and Southeast Asian populations
- Causes: Oxidative stress triggers hemolysis, certain medications (antimalarials, sulfonamides) can exacerbate hemolysis
- Symptoms: Hemolytic anemia, jaundice, and dark urine
- Diagnosis: Enzyme assay and fluorescent spot test
- Treatment: Avoidance of triggering medications and supportive care (hydration, transfusions)
Hereditary Spherocytosis (HS)
- Inherited disorder affecting RBC membrane, leading to spherocytosis and hemolytic anemia
- Autosomal dominant inheritance, most common in people of European descent
- Causes: Mutations in RBC membrane protein genes (e.g., ANK1, SPTA1)
- Symptoms: Hemolytic anemia, jaundice, and splenomegaly
- Diagnosis: Osmotic fragility test and Eosin-5'-maleimide (EMA) binding test
- Treatment: Splenectomy and supportive care (transfusions, iron chelation)
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Description
Test your knowledge of Autoimmune Hemolytic Anemia, a type of hemolytic disorder caused by autoantibody-mediated destruction of red blood cells. Learn about its types, causes, symptoms, and diagnosis.
