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Questions and Answers
What characterizes haemolytic anaemia?
Which of the following is an intrinsic cause of haemolytic anaemia?
Which laboratory findings are indicative of haemolysis?
What can happen if haemolytic anaemia is not managed promptly?
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Which type of autoantibody is involved in warm antibody autoimmune haemolytic anaemia (AIHA)?
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Which factor can be associated with cold antibody AIHA?
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What are spherocytes commonly seen in?
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Which condition can indicate increased destruction of red blood cells?
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What is a characteristic finding in warm autoimmune hemolytic anemia (AIHA)?
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What is the inheritance pattern of sickle cell anemia?
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Which factor can precipitate sickling in sickle cell disease?
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What is the typical lifespan of sickle-shaped red blood cells?
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Which of the following is not a complication associated with sickle cell disease?
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What occurs to hemoglobin in the deoxygenated state in sickle cell disease?
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Which of the following symptoms is associated with hemolytic anemia?
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What is the primary issue that leads to increased blood viscosity in sickle cell disease?
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What is a key characteristic of haemolytic anaemia?
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Which laboratory test is indicative of haemolysis?
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What can be a severe consequence of rapid haemolysis?
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What causes elevated bilirubin levels in haemolytic anaemia?
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What is a common hallmark of autoimmune haemolytic anaemia?
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Which of the following is NOT a classification of haemolytic anaemia?
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What is the primary trigger for cold antibody autoimmune haemolytic anaemia?
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What is a common cause of intrinsic haemolytic anaemia?
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What type of antibodies are associated with warm autoimmune hemolytic anemia (AIHA)?
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Which complication is commonly associated with sickle cell disease?
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What is the primary genetic alteration in sickle cell disease?
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What is the expected lifespan of sickle-shaped red blood cells?
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Which of the following best describes the structural change that occurs in sickle cell disease?
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What factor can lead to vascular occlusion in sickle cell disease?
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What is a key aspect of the history that should be taken in patients suspected of having hemolytic anemia?
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Which of the following is a known precipitating factor for sickling events in sickle cell disease?
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What is a characteristic of rapid haemolysis in haemolytic anaemia?
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What type of antibodies is associated with cold antibody autoimmune haemolytic anaemia?
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Which of the following accurately describes a cause classified as extrinsic to red cells in haemolytic anaemia?
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What laboratory findings are associated with autoimmune haemolytic anaemia?
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In sickle cell disease, which complication is NOT typically associated?
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What metabolic consequence arises primarily from the destruction of red blood cells in haemolytic anaemia?
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Which form of haemolytic anaemia is characterized by antibodies that react at body temperature?
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Which condition is characterized by intrinsic abnormalities affecting the red blood cells?
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What is the primary cause of vascular occlusion in sickle cell disease?
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Which symptom would most likely indicate a complication in a patient with sickle cell disease?
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What inheritance pattern is observed in sickle cell trait?
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What is a consequence of the structural change in red blood cells due to sickle cell disease?
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In which scenario would sickling events most likely be triggered in patients with sickle cell disease?
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Which of these conditions is commonly associated with chronic hemolytic anemia?
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Which factor would not contribute to the crisis in sickle cell disease?
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What genetic alteration is responsible for sickle cell disease?
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Study Notes
Haemolytic Anaemia
- Haemolytic anaemia is a condition where mature red blood cells are destroyed at a faster rate than they can be produced.
- Haemolysis, the destruction of red blood cells, can lead to high levels of bilirubin and LDH in the bloodstream.
- Haemolytic anaemia can be asymptomatic until haemoglobin levels are dangerously low.
- Rapid haemolysis can be life-threatening and requires emergency medical attention.
Causes
-
Intrinsic to red cells:
- Hereditary spherocytosis: Hereditary blood disorder that causes red blood cells to be spherical (spherocytes) instead of biconcave, leading to increased destruction.
- Sickle cell disease: Genetic disorder where red blood cells adopt a sickle shape, resulting in increased fragility and reduced lifespan.
- Thalassaemia: Group of inherited blood disorders affecting the production of haemoglobin, causing red blood cell abnormalities and increased destruction
- G-6PD deficiency: Inherited enzyme deficiency that leads to red blood cell damage in response to certain oxidative stressors.
-
Extrinsic to red cells:
-
Immune: Autoimmune haemolytic anaemia (AIHA), where the body's immune system attacks its own red blood cells.
- Warm antibody AIHA: Caused by IgG autoantibodies
- Cold antibody AIHA: Caused by IgM autoantibodies
- Mechanical: Damage to red blood cells from physical forces like artificial heart valves, or traumatic injuries.
-
Immune: Autoimmune haemolytic anaemia (AIHA), where the body's immune system attacks its own red blood cells.
Autoimmune Haemolytic Anaemia
- Warm antibody AIHA is often associated with conditions like lymphoma, Chronic Lymphocytic Leukaemia (CLL), and collagen vascular diseases.
- Cold antibody AIHA is seen in conditions like cold agglutinin disease, mycoplasma infections, and Epstein-Barr virus infections.
-
Diagnosis of AIHA:
- Increased reticulocyte count (immature red blood cells)
- Elevated LDH (lactate dehydrogenase) levels
- Increased indirect bilirubin
- Peripheral blood smear may show spherocytes and fragmented RBCs
-
Positive Direct Coombs Test (DAT): Indicates the presence of antibodies on the surface of red blood cells.
- Warm AIHA: Positive for IgG and/or C3
- Cold AIHA: Positive for C3, but negative for IgG
Clinical History in Haemolytic Anaemia
- Symptoms: Similar to other anaemia causes but with a shorter onset.
- Infectious Symptoms: Cough, fever, recent contact with ill individuals.
- Medication History: New drugs or over-the-counter medication use
- Travel History: Recent travel to areas where malaria is prevalent
- Family History: Known family history of haemolytic anaemia or inherited blood disorders.
- Previous Transfusions: Crucial information to acquire, as a history of blood transfusions can indicate past episodes of haemolytic anaemia.
Clinical Examination
- Physical examination may reveal signs of anaemia, including pallor, jaundice, and splenomegaly (enlarged spleen).
Inherited Haemoglobin Defects
-
Sickle Cell Disease: Caused by a mutation in the beta-globin gene, resulting in the production of abnormal haemoglobin S (HbS).
- Sickle cell trait: Carriers have one copy of the sickle cell gene (HbS) and one copy of the normal gene (HbA), and usually do not experience symptoms.
- Sickle cell anaemia: Individuals inherit two copies of the sickle cell gene, resulting in significant clinical consequences.
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Thalassaemia: A group of genetic disorders affecting the production of alpha or beta globin chains, which are needed to form haemoglobin.
- Thalassaemia major: Severe form requiring lifelong blood transfusions
- Thalassaemia minor: Milder form, often asymptomatic or with minimal signs and symptoms.
Sickle Cell Disease
- The sickle haemoglobin (HbS) under low oxygen conditions crystallizes, deforming the red blood cell into a sickle shape.
- Sickle-shaped red blood cells have a shorter lifespan (10-20 days) compared to normal red blood cells (120 days).
- These rigid cells have difficulty passing through small blood vessels, leading to vascular occlusion, tissue hypoxia, and infarction (tissue death).
Sickling Triggers
- Hypoxia: Low oxygen levels
- Acidosis: High acidity in the blood
- Hypotension: Low blood pressure
- Infection: Bacterial or viral infections
- Dehydration: Fluid loss
- Hypothermia: Low body temperature
Complications of Sickle Cell Disease
- Osteomyelitis: Infected bone, particularly in those with sickle cell disease, due to frequent vaso-occlusion.
- Gallstones: Increased bilirubin breakdown leads to gallstone formation.
- Renal Failure: Chronic vaso-occlusive events in the kidneys can lead to progressive damage and renal failure.
- Cardiac Failure: Increased workload on the heart due to chronic anaemia and vaso-occlusion can result in heart failure.
- Chronic Leg Ulcer: Poor circulation and vaso-occlusion in the legs can lead to chronic ulcer formations.
Haemolytic Anaemia
- A condition where mature red blood cells are destroyed more rapidly than they can be produced.
- This leads to the release of red blood cell contents into the bloodstream, resulting in elevated bilirubin and LDH levels.
- Haemolytic anaemia can be asymptomatic until haemoglobin levels become dangerously low.
- Rapid haemolysis can be life-threatening and requires emergency management.
Causes of Haemolytic Anaemia
-
Intrinsic to red cells:
- Hereditary spherocytosis
- Sickle cell disease
- Thalassaemia
- G-6PD deficiency
-
Extrinsic to red cells:
- Immune
- Mechanical
Autoimmune Haemolytic Anaemia (AIHA)
-
Warm antibody AIHA:
- Caused by an IgG autoantibody.
- Associated with conditions like lymphoma, CLL, and collagen vascular diseases.
-
Cold antibody AIHA:
- Caused by an IgM autoantibody.
- Seen in cold agglutinin diseases, Mycoplasma, and Epstein-Barr virus infections.
-
Diagnosis:
- Reticulocytosis, elevated LDH, and indirect hyperbilirubinaemia.
- Peripheral blood smear may show spherocytes and fragmented RBCs.
- Positive direct Coombs test (DAT):
- Warm AIHA: IgG+ and/or C3+
- Cold AIHA: IgG- and C3+
History Taking in Haemolytic Anaemia
- Symptoms: Similar to other causes of anaemia, but with a shorter onset.
- Infectious symptoms: Cough, fevers, recent contact with ill individuals.
- Recent drug use: New medications or over-the-counter drugs.
- Recent travel: Potential for malaria exposure.
- Family history: Inherited haemolytic anaemias.
- Previous transfusions: Important to inquire about, even distant or unknown history.
Inherited Haemoglobin Defects
- Abnormal genetic code in haemoglobin.
-
Sickle Cell Disease:
- Mutation in haemoglobin causing increased HbS with red blood cell aggregation at low oxygen tensions.
-
Thalassaemia:
- Globin chains are normal but synthesis rate is reduced.
- Accumulation of abnormal chains leads to structural defects.
Sickle Cell Disease
- Sickle Cell Anaemia: Inherited HBS gene and another abnormal β chain variant.
- Sickle Cell Trait: Inherited one gene encoding HBS.
-
Pathophysiology:
- HbS in deoxygenated state is less soluble than HbA.
- Insoluble chains crystallise in red cells, distorting the membrane and making the cell crescent-shaped.
- Sickle RBCs have a shorter lifespan (10-20 days).
- Deformed cells are more rigid and cannot pass through microcirculation, causing vascular occlusion.
- Structural change and increased blood viscosity lead to venous stasis, local obstruction, tissue hypoxia, more sickling, and tissue infarction.
Sickle Cell Disease: Precipitating Factors
- Spontaneous sickling can occur.
- Precipitating factors:
- Hypoxia
- Acidosis
- Hypotension
- Infection
- Dehydration
- Hypothermia
Other Complications of Sickle Cell Disease
- Osteomyelitis (infected bones): Due to vascular occlusion and impaired blood flow.
- Gallstones: Due to increased bilirubin production.
- Renal failure: Due to vascular occlusion and damage to renal tubules.
- Cardiac failure: Due to chronic anaemia and increased workload on the heart.
- Chronic leg ulcers: Due to impaired blood flow and tissue damage.
Haemolytic Anaemia
- Mature red blood cells are destroyed faster than they can be produced.
- Elevated bilirubin and LDH levels in the bloodstream can indicate haemolysis.
- Rapid haemolysis can be life-threatening and requires emergency treatment.
Causes of Haemolytic Anaemia
-
Intrinsic to red cells:
- Hereditary spherocytosis
- Sickle cell disease
- Thalassaemia
- G-6PD deficiency
-
Extrinsic to red cells:
- Immune
- Mechanical
Autoimmune Haemolytic Anaemia (AIHA)
-
Warm antibody AIHA: caused by IgG autoantibody
- Associated with lymphoma, CLL, and collagen vascular diseases.
-
Cold antibody AIHA: caused by IgM autoantibody
- Seen in cold agglutinin diseases, mycoplasma, and EB virus
-
Diagnosis features:
- Reticulocytosis
- Elevated LDH
- Indirect hyperbilirubinaemia
- Spherocytes and fragmented RBCs on peripheral blood smear
- Positive Direct Coombs Test (DAT)
- Warm AIHA: IgG+ and/or C3+
- Cold AIHA: IgG- and C3+
Sickle Cell Disease
- Sickle Cell Anaemia: Inheritance of HBS and another abnormal β chain variant
- Sickle Cell Trait: Inheritance of one gene encoding for HBS
-
Pathogenesis:
- Sickle cell mutation causes increased HbS which aggregates at low oxygen tensions.
- Deoxygenated HbS is less soluble than HbA and forms crystals within red cells.
- Cells become crescent shaped and are more rigid.
- They are fragile and have a shortened lifespan (10-20 days).
- Deformed cells get trapped in the microcirculation, causing vascular occlusion.
- This leads to tissue hypoxia, increased sickling, and tissue infarction.
-
Clinical presentation:
- Chronic haemolytic anaemia
- Recurrent episodes of severe pain in the limbs
- Precipitated by: hypoxia, acidosis, hypotension, infection, dehydration, hypothermia
Complications of Sickel Cell Disease
- Osteomyelitis (infected bones)
- Gallstones
- Renal failure
- Cardiac failure
- Chronic leg ulcers
Thalassaemia
- Globin chains are normal, but the rate of synthesis is reduced.
- Accumulation of abnormal chains causes structural defects
- Thalassaemia major and minor
History Taking in Haemolytic Anaemia
- Symptoms: similar to other causes of anaemia but with a faster onset.
- Infectious symptoms: cough, fevers, contact with ill individuals.
- Recent new drugs: medication or over-the-counter medications.
- Recent travel: potential for malaria exposure.
- Family history: check for sickle cell disease or thalassaemia.
-
Previous transfusions:
- Very important to check even for unknown or distant history
- Check with lab for previous transfusion records.
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Description
This quiz explores haemolytic anaemia, a condition characterized by the rapid destruction of red blood cells. You'll learn about its causes, symptoms, and the implications of haemolysis on health. Test your knowledge on hereditary disorders and their relationship to this serious health condition.
