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Questions and Answers
What type of lattice is formed by spectrin tetramers, hexamers, or double tetramers?
What type of lattice is formed by spectrin tetramers, hexamers, or double tetramers?
Which molecule is bound to spectrin filaments approximately 80 nm from their distal ends?
Which molecule is bound to spectrin filaments approximately 80 nm from their distal ends?
What is contained within cross-linking junctional complexes?
What is contained within cross-linking junctional complexes?
Which technique is used for separating the proteins listed in the properties of selected red cell membrane proteins?
Which technique is used for separating the proteins listed in the properties of selected red cell membrane proteins?
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What is the primary focus of the table about red cell membrane proteins?
What is the primary focus of the table about red cell membrane proteins?
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What abnormality is noted in the peripheral blood smear of a patient with hereditary pyropoikilocytosis (HPP)?
What abnormality is noted in the peripheral blood smear of a patient with hereditary pyropoikilocytosis (HPP)?
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Which finding is characteristic of Southeast Asian ovalocytosis (SAO)?
Which finding is characteristic of Southeast Asian ovalocytosis (SAO)?
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In hereditary elliptocytosis (HE) and hereditary pyropoikilocytosis (HPP), what is the typical finding regarding MCHC in infants?
In hereditary elliptocytosis (HE) and hereditary pyropoikilocytosis (HPP), what is the typical finding regarding MCHC in infants?
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What is the osmotic fragility test result typically observed in patients with hereditary elliptocytosis (HE)?
What is the osmotic fragility test result typically observed in patients with hereditary elliptocytosis (HE)?
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What is the typical MCV result in a patient with hereditary pyropoikilocytosis (HPP)?
What is the typical MCV result in a patient with hereditary pyropoikilocytosis (HPP)?
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What does an RPI greater than 2.5-3.0 indicate?
What does an RPI greater than 2.5-3.0 indicate?
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What is the significance of a reticulocyte production index (RPI)?
What is the significance of a reticulocyte production index (RPI)?
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What is indicated by the presence of Prussian blue-positive deposits of hemosiderin in urine?
What is indicated by the presence of Prussian blue-positive deposits of hemosiderin in urine?
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When assessing red blood cell production, what would a reduction in the myeloid-to-erythroid (M:E) ratio from 3-4:1 to 1:1 suggest?
When assessing red blood cell production, what would a reduction in the myeloid-to-erythroid (M:E) ratio from 3-4:1 to 1:1 suggest?
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Which of the following tests is used to assess the osmotic fragile nature of red blood cells?
Which of the following tests is used to assess the osmotic fragile nature of red blood cells?
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In acute intravascular hemolysis, what would you expect to see in urine samples?
In acute intravascular hemolysis, what would you expect to see in urine samples?
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What conclusion can be drawn if reticulocyte maturation times are found to be prolonged?
What conclusion can be drawn if reticulocyte maturation times are found to be prolonged?
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Which of the following statements about hemoglobinuria is accurate?
Which of the following statements about hemoglobinuria is accurate?
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What is the primary defect leading to spherocytes in hereditary spherocytosis?
What is the primary defect leading to spherocytes in hereditary spherocytosis?
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What percentage of RBCs can be uniform elliptocytes in mild hereditary elliptocytosis (HE)?
What percentage of RBCs can be uniform elliptocytes in mild hereditary elliptocytosis (HE)?
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What lab finding indicates severe cases of hereditary elliptocytosis (HE) and hereditary pyropoikilocytosis (HPP)?
What lab finding indicates severe cases of hereditary elliptocytosis (HE) and hereditary pyropoikilocytosis (HPP)?
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In hereditary pyropoikilocytosis (HPP), cells show increased sensitivity to what condition?
In hereditary pyropoikilocytosis (HPP), cells show increased sensitivity to what condition?
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What feature is typically seen in spherocytic hereditary elliptocytosis (HE)?
What feature is typically seen in spherocytic hereditary elliptocytosis (HE)?
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What is the morphology of peripheral smear in hereditary elliptocytosis (HE) during a mild case?
What is the morphology of peripheral smear in hereditary elliptocytosis (HE) during a mild case?
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In stomatocytic hereditary elliptocytosis, how do the elliptocytes appear?
In stomatocytic hereditary elliptocytosis, how do the elliptocytes appear?
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Which condition is associated with increased thermal sensitivity and misshapen budding in red blood cells?
Which condition is associated with increased thermal sensitivity and misshapen budding in red blood cells?
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What is the morphology described in the peripheral blood smear for Hst?
What is the morphology described in the peripheral blood smear for Hst?
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In the context of red cell indices, how does MCHC differ between Hst and Hxe?
In the context of red cell indices, how does MCHC differ between Hst and Hxe?
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What is a characteristic finding in Osmotic fragility tests for Hst?
What is a characteristic finding in Osmotic fragility tests for Hst?
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Hereditary Acanthocytosis is inherited in which manner?
Hereditary Acanthocytosis is inherited in which manner?
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What is a key feature associated with the condition of Hereditary Acanthocytosis?
What is a key feature associated with the condition of Hereditary Acanthocytosis?
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Which enzyme deficiency is the most common in the hexose monophosphate shunt?
Which enzyme deficiency is the most common in the hexose monophosphate shunt?
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What is the main source of energy for nucleated red cells?
What is the main source of energy for nucleated red cells?
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What is a consequence of G6PD deficiency regarding glutathione levels?
What is a consequence of G6PD deficiency regarding glutathione levels?
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Which condition is most consistent with hemolytic episodes caused by anti-malarial drugs in a patient with congenital non-spherocytic hemolytic anemia?
Which condition is most consistent with hemolytic episodes caused by anti-malarial drugs in a patient with congenital non-spherocytic hemolytic anemia?
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What are the red cell inclusions in hemolytic anemia primarily caused by?
What are the red cell inclusions in hemolytic anemia primarily caused by?
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Hereditary pyropoikilocytosis is characterized by the presence of which type of red blood cells?
Hereditary pyropoikilocytosis is characterized by the presence of which type of red blood cells?
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What symptom indicates a potential hemolytic event in a patient taking primaquine?
What symptom indicates a potential hemolytic event in a patient taking primaquine?
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Anti-malarial drugs can precipitate hemolysis in which of the following conditions?
Anti-malarial drugs can precipitate hemolysis in which of the following conditions?
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Which of the following is not typically associated with congenital hemolytic anemias?
Which of the following is not typically associated with congenital hemolytic anemias?
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What is the main mechanism behind hemolytic episodes precipitated by anti-malarial drugs in sensitive individuals?
What is the main mechanism behind hemolytic episodes precipitated by anti-malarial drugs in sensitive individuals?
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Which of the following is a characteristic finding in hereditary pyropoikilocytosis?
Which of the following is a characteristic finding in hereditary pyropoikilocytosis?
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Study Notes
Hemolytic Anemia
- Hemolytic anemia (HA) is a group of normocytic normochromic anemias characterized by premature destruction of red blood cells (RBCs) or shortened RBC lifespan.
- Bone marrow (BM) often increases output 6-8-fold to compensate for the loss of RBCs. This corresponds to a normal RBC lifespan of 15-20 days.
Classification of Hemolytic Anemia
-
Intracorpuscular defects
- Hereditary defects: Defects in RBC membrane, enzymes, hemoglobinopathies, & thalassemia syndrome.
- Acquired defects: Paroxysmal nocturnal hemoglobinuria (PNH)
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Extracorpuscular defects
- Immune hemolytic anemia
- Infections
- Exposure to chemicals and toxins
- Exposure to physical agents
- Microangiopathic and macroangiopathic hemolytic anemias
- Splenic sequestration (hypersplenism)
- General systemic disorders (where hemolysis isn't the primary feature of the disorder)
Diagnosis of Hemolytic Anemia (HA)
-
Tests reflecting increased red cell destruction:
- Serum un-conjugated (indirect) bilirubin
- Hemoglobinemia
- Hemoglobinuria
- Hemosiderinuria
- Methemalbumin in the blood (Schumm's test)
- Methemoglobinemia
- Serum hemopexin
- Serum haptoglobin
- Urine urobilinogen and fecal stercobilinogen increased
Degradation of Hb After Intra- & Extra-vascular Hemolysis
- Breakdown of hemoglobin (Hb), releasing iron, globin, and bilirubin (which enters the liver) is dependent on whether hemolysis is intravascular or extravascular.
Tests Reflecting Increased Red Cell Production
- Reticulocyte count: Measures immature RBCs
- Reticulocyte production index (RPI): Adjusts reticulocyte count to account for RBC maturation time in relation to hematocrit.
Red Cell Morphology
- Red blood cell deformability depends on biconcave disc shape maximizing surface area-to-volume ratio
- Cytoplasmic viscosity determined by mean cell hemoglobin concentration (MCHC)
- Viscoelastic properties depend on membrane skeleton integrity.
RBC Membrane Proteins
- Integral Proteins: Glycophorin A, B, C, D, anion exchange channel protein (band 3).
- Peripheral Proteins: Spectrin, actin (band 5), ankyrin (band 2.1), band 4.1 & 4.2, adducin, p55.
- Mutations in these proteins can lead to various hereditary hemolytic anemias.
Hereditary Defects of the Red Cell Membrane
- Genetic mutations affect the RBC membrane, impacting shape, function, and lifespan.
- Hereditary spherocytosis (HS)
- Hereditary elliptocytosis (HE), including hereditary pyropoikilocytosis (HPP) and Southeast Asian ovalocytosis (SAO).
- Hereditary stomatocytosis
- Hereditary xerocytosis
- Hereditary acanthocytosis
Assessment Questions
- Questions on hemolytic anemia characteristics cover common diagnoses, including hereditary spherocytosis, elliptocytosis, stomatocytosis, and paroxysmal nocturnal hemoglobinuria.
- Further questions assess understanding of specific hemolytic anemias like G6PD deficiency and pyruvate kinase (PK) deficiency.
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Description
Test your knowledge about red cell membrane proteins and disorders such as hereditary elliptocytosis and pyropoikilocytosis. This quiz covers key concepts, techniques, and typical findings related to red blood cell membranes. Explore the structure of spectrin and other essential components that influence red blood cell shape and function.
