Podcast
Questions and Answers
What morphological characteristic defines a mature erythrocyte?
What morphological characteristic defines a mature erythrocyte?
- Spherical shape with a granular cytoplasm
- Irregular shape with multiple nuclei
- Large cell with a dense, dark-staining nucleus
- Biconcave disc with a central pallor (correct)
What process is defined as the production of erythrocytes?
What process is defined as the production of erythrocytes?
- Erythropoiesis (correct)
- Leukopoiesis
- Thrombopoiesis
- Granulopoiesis
Where does erythropoiesis primarily occur?
Where does erythropoiesis primarily occur?
- In the spleen around lymphocytes
- In the thymus around thymocytes
- In erythroblastic islands (correct)
- In the liver within Kupffer cells
What role does the macrophage play within the erythroblastic island?
What role does the macrophage play within the erythroblastic island?
What is the typical lifespan of a mature erythrocyte in circulation?
What is the typical lifespan of a mature erythrocyte in circulation?
Which of the following best describes the primary function of hemoglobin within a mature erythrocyte?
Which of the following best describes the primary function of hemoglobin within a mature erythrocyte?
A newborn infant has a reticulocyte count of 5%. How does this compare to adult levels?
A newborn infant has a reticulocyte count of 5%. How does this compare to adult levels?
Why is it necessary to correct the reticulocyte count in patients with anemia?
Why is it necessary to correct the reticulocyte count in patients with anemia?
Which of the following trace minerals are required for normal erythropoiesis?
Which of the following trace minerals are required for normal erythropoiesis?
What is a primary function of mature erythrocytes?
What is a primary function of mature erythrocytes?
What metabolic change occurs in erythrocytes as they age?
What metabolic change occurs in erythrocytes as they age?
What is the range of normal reticulocyte count in adults?
What is the range of normal reticulocyte count in adults?
What is the average diameter of a mature erythrocyte?
What is the average diameter of a mature erythrocyte?
Which metabolic process is used by erythrocytes to metabolize glucose?
Which metabolic process is used by erythrocytes to metabolize glucose?
What is the purpose of calculating the Reticulocyte Production Index (RPI)?
What is the purpose of calculating the Reticulocyte Production Index (RPI)?
Which of the following conditions is associated with an increase in erythropoietin production?
Which of the following conditions is associated with an increase in erythropoietin production?
Besides the Embden-Meyerhof-Parnas pathway (anaerobic glycolysis), which of the following is a supplementary pathway involved in erythrocyte metabolism?
Besides the Embden-Meyerhof-Parnas pathway (anaerobic glycolysis), which of the following is a supplementary pathway involved in erythrocyte metabolism?
What is the primary factor that causes the appearance of an elevated hematocrit in RELATIVE polycythemia?
What is the primary factor that causes the appearance of an elevated hematocrit in RELATIVE polycythemia?
Which process leads to a loss of surface area in a maturing reticulocyte?
Which process leads to a loss of surface area in a maturing reticulocyte?
Defective DNA synthesis in megaloblastic maturation primarily affects which cellular process?
Defective DNA synthesis in megaloblastic maturation primarily affects which cellular process?
In the maturation defect called megaloblastic maturation, how does the development of the nucleus compare to that of the cytoplasm?
In the maturation defect called megaloblastic maturation, how does the development of the nucleus compare to that of the cytoplasm?
What happens to the amount of tubulin and actin within the membrane as a reticulocyte matures into an erythrocyte?
What happens to the amount of tubulin and actin within the membrane as a reticulocyte matures into an erythrocyte?
Which of the following is not a major change that occurs as a reticulocyte matures into an erythrocyte?
Which of the following is not a major change that occurs as a reticulocyte matures into an erythrocyte?
A patient residing at a high altitude is found to have an elevated red blood cell count. Which mechanism is most likely responsible for this increase?
A patient residing at a high altitude is found to have an elevated red blood cell count. Which mechanism is most likely responsible for this increase?
Which hematological condition is characterized by an increased concentration of erythrocytes in circulating blood beyond normal levels for gender and age?
Which hematological condition is characterized by an increased concentration of erythrocytes in circulating blood beyond normal levels for gender and age?
What is the primary structure of the erythrocyte cellular membrane?
What is the primary structure of the erythrocyte cellular membrane?
Which of the following is a potential cause of secondary polycythemia?
Which of the following is a potential cause of secondary polycythemia?
What factor does not affect the shape changes of a mature red blood cell?
What factor does not affect the shape changes of a mature red blood cell?
What is the most significant factor leading to prolonged interphase and mitotic division phases in cells with megaloblastic maturation?
What is the most significant factor leading to prolonged interphase and mitotic division phases in cells with megaloblastic maturation?
Which of the following is NOT a major component of a mature erythrocyte cytoskeleton?
Which of the following is NOT a major component of a mature erythrocyte cytoskeleton?
Which of the following describes a change that occurs in the RBC membrane as it ages?
Which of the following describes a change that occurs in the RBC membrane as it ages?
In early erythrocyte development, what is an important role of cytoplasmic enzymes regarding intracellular ion concentrations?
In early erythrocyte development, what is an important role of cytoplasmic enzymes regarding intracellular ion concentrations?
What is the consequence if metabolic pathways in erythrocytes are blocked or functioning inadequately?
What is the consequence if metabolic pathways in erythrocytes are blocked or functioning inadequately?
Which function is NOT critical for maintaining the integrity and function of erythrocytes?
Which function is NOT critical for maintaining the integrity and function of erythrocytes?
What is the primary function of REDUCED glutathione in erythrocytes?
What is the primary function of REDUCED glutathione in erythrocytes?
Deficiencies in which of the following coenzymes can be classified as a metabolic defect in erythrocytes?
Deficiencies in which of the following coenzymes can be classified as a metabolic defect in erythrocytes?
Which enzyme deficiency directly impairs the conversion of glucose-6-phosphate (G6P) to 6-phosphogluconate (6PG)?
Which enzyme deficiency directly impairs the conversion of glucose-6-phosphate (G6P) to 6-phosphogluconate (6PG)?
What is the primary outcome of the Embden-Meyerhof-Parnas pathway in erythrocytes?
What is the primary outcome of the Embden-Meyerhof-Parnas pathway in erythrocytes?
Failure to reduce glutathione leads to which of the following consequences in erythrocytes?
Failure to reduce glutathione leads to which of the following consequences in erythrocytes?
Which pathway is responsible for maintaining hemoglobin in a reduced state to prevent its oxidation?
Which pathway is responsible for maintaining hemoglobin in a reduced state to prevent its oxidation?
Under which conditions does the Luebering-Rapoport pathway become particularly important for optimal oxygen transport?
Under which conditions does the Luebering-Rapoport pathway become particularly important for optimal oxygen transport?
What is the role of Pyruvate Kinase (PK) in the Embden-Meyerhof-Parnas glycolytic pathway?
What is the role of Pyruvate Kinase (PK) in the Embden-Meyerhof-Parnas glycolytic pathway?
Which of the following is a potential cause of sideroblastic anemia?
Which of the following is a potential cause of sideroblastic anemia?
In disorders of globin synthesis, what happens when porphyrin synthesis is impaired?
In disorders of globin synthesis, what happens when porphyrin synthesis is impaired?
What accumulates in the cytoplasm of cells when globin production is deficient?
What accumulates in the cytoplasm of cells when globin production is deficient?
Which of the following is an embryonic hemoglobin?
Which of the following is an embryonic hemoglobin?
Until approximately how many weeks of gestation do embryonic hemoglobins persist?
Until approximately how many weeks of gestation do embryonic hemoglobins persist?
A defect in the rate of synthesis of one or more globulin polypeptide chains in the hemoglobin molecule can lead to what class of diseases?
A defect in the rate of synthesis of one or more globulin polypeptide chains in the hemoglobin molecule can lead to what class of diseases?
In sideroblastic anemia associated with malignant marrow disorders, which of the following is a possible condition?
In sideroblastic anemia associated with malignant marrow disorders, which of the following is a possible condition?
What is the primary consequence of sulfhemoglobin formation?
What is the primary consequence of sulfhemoglobin formation?
At what percentage of methemoglobin level does cyanosis typically occur?
At what percentage of methemoglobin level does cyanosis typically occur?
In sickle cell anemia Hemoglobin S, what amino acid substitution occurs at the 6th position of the β chain of the hemoglobin molecule?
In sickle cell anemia Hemoglobin S, what amino acid substitution occurs at the 6th position of the β chain of the hemoglobin molecule?
Which of the following hemoglobins is NOT typically separated using alkaline electrophoresis?
Which of the following hemoglobins is NOT typically separated using alkaline electrophoresis?
What is the underlying principle of alkaline electrophoresis in separating hemoglobin molecules?
What is the underlying principle of alkaline electrophoresis in separating hemoglobin molecules?
Which of the following is a characteristic of 'fast hemoglobins' in alkaline electrophoresis?
Which of the following is a characteristic of 'fast hemoglobins' in alkaline electrophoresis?
What is a common feature of 'slow hemoglobins' during alkaline electrophoresis?
What is a common feature of 'slow hemoglobins' during alkaline electrophoresis?
A patient with sickle cell trait has the genotype SA. What does this indicate?
A patient with sickle cell trait has the genotype SA. What does this indicate?
When an erythrocyte is phagocytized by macrophages in the spleen, what components result from the disassembly of its hemoglobin molecules?
When an erythrocyte is phagocytized by macrophages in the spleen, what components result from the disassembly of its hemoglobin molecules?
What is the role of transferrin in the extravascular catabolism of erythrocytes?
What is the role of transferrin in the extravascular catabolism of erythrocytes?
What typically happens to globin after extravascular catabolism of erythrocytes?
What typically happens to globin after extravascular catabolism of erythrocytes?
What is the immediate fate of hemoglobin released directly into the bloodstream during intravascular destruction?
What is the immediate fate of hemoglobin released directly into the bloodstream during intravascular destruction?
What is the primary purpose of MOLECULAR testing in the analysis of hemoglobin?
What is the primary purpose of MOLECULAR testing in the analysis of hemoglobin?
What change occurs to the erythrocyte membrane as it ages?
What change occurs to the erythrocyte membrane as it ages?
In aged erythrocytes, what happens to the activity of glycolytic enzymes?
In aged erythrocytes, what happens to the activity of glycolytic enzymes?
What proportion of erythrocytic destruction is normally attributed to intravascular destruction?
What proportion of erythrocytic destruction is normally attributed to intravascular destruction?
Which chromosome contains the Alpha Globin Locus which governs alpha chain production in adults?
Which chromosome contains the Alpha Globin Locus which governs alpha chain production in adults?
Which type of hemoglobin comprises two alpha and two beta globin chains?
Which type of hemoglobin comprises two alpha and two beta globin chains?
What are porphyrias associated with?
What are porphyrias associated with?
Which of these globin chains are produced by the Beta Globin Locus on chromosome 11? (Two answers)
Which of these globin chains are produced by the Beta Globin Locus on chromosome 11? (Two answers)
Which of the following hemoglobin types is characterized by its composition of polypeptide chains?
Which of the following hemoglobin types is characterized by its composition of polypeptide chains?
During the chloride shift, what happens to free bicarbonate in RBCs?
During the chloride shift, what happens to free bicarbonate in RBCs?
How is carbon dioxide transported in the blood?
How is carbon dioxide transported in the blood?
Where is heme primarily formed in the body?
Where is heme primarily formed in the body?
What role does transferrin play in hemoglobin synthesis?
What role does transferrin play in hemoglobin synthesis?
What is the primary outcome of iron entry into immature erythrocytes?
What is the primary outcome of iron entry into immature erythrocytes?
What process converts bicarbonate back into carbon dioxide in the lungs?
What process converts bicarbonate back into carbon dioxide in the lungs?
Excess non-heme iron accumulates in the cytoplasm of immature erythrocytes as what?
Excess non-heme iron accumulates in the cytoplasm of immature erythrocytes as what?
What governs the structure and production of globin in erythrocytes?
What governs the structure and production of globin in erythrocytes?
What is the effect of 2,3-DPG binding to deoxyhemoglobin?
What is the effect of 2,3-DPG binding to deoxyhemoglobin?
What occurs in the lungs when hemoglobin binds to oxygen?
What occurs in the lungs when hemoglobin binds to oxygen?
What happens to 2,3-DPG levels in red blood cells during persistent tissue hypoxia?
What happens to 2,3-DPG levels in red blood cells during persistent tissue hypoxia?
What does a shift to the left on the oxyhemoglobin dissociation curve indicate?
What does a shift to the left on the oxyhemoglobin dissociation curve indicate?
What does the $P_{50}$ value represent in the context of the oxyhemoglobin dissociation curve?
What does the $P_{50}$ value represent in the context of the oxyhemoglobin dissociation curve?
Apart from direct and indirect transport by erythrocytes, how else is carbon dioxide carried to the lungs?
Apart from direct and indirect transport by erythrocytes, how else is carbon dioxide carried to the lungs?
In the indirect erythrocyte mechanism of carbon dioxide transport, what enzyme catalyzes the transformation of carbon dioxide into carbonic acid?
In the indirect erythrocyte mechanism of carbon dioxide transport, what enzyme catalyzes the transformation of carbon dioxide into carbonic acid?
Approximately what proportion of carbon dioxide is removed via the indirect erythrocyte mechanism?
Approximately what proportion of carbon dioxide is removed via the indirect erythrocyte mechanism?
How do changes in the oxygen affinity of hemoglobin facilitate oxygen transport?
How do changes in the oxygen affinity of hemoglobin facilitate oxygen transport?
A shift to the right on the oxygen dissociation curve indicates what?
A shift to the right on the oxygen dissociation curve indicates what?
Which of the following erythrocyte inclusions can ONLY be visualized using a supravital stain?
Which of the following erythrocyte inclusions can ONLY be visualized using a supravital stain?
Fine basophilic stippling is characterized by which of the following features?
Fine basophilic stippling is characterized by which of the following features?
Coarse basophilic stippling (sometimes called "punctate" stippling) is considered more serious than fine basophilic stippling due to its association with:
Coarse basophilic stippling (sometimes called "punctate" stippling) is considered more serious than fine basophilic stippling due to its association with:
What are the granules found in basophilic stippling composed of?
What are the granules found in basophilic stippling composed of?
Which clinical condition is least likely to be associated with basophilic stippling?
Which clinical condition is least likely to be associated with basophilic stippling?
What does the Mean Corpuscular Hemoglobin (MCH) express?
What does the Mean Corpuscular Hemoglobin (MCH) express?
What is the normal range for Mean Corpuscular Volume (MCV) in femtoliters (fL)?
What is the normal range for Mean Corpuscular Volume (MCV) in femtoliters (fL)?
If a patient's Mean Corpuscular Hemoglobin Concentration (MCHC) is reported as 30%, how would this be interpreted in relation to the normal range?
If a patient's Mean Corpuscular Hemoglobin Concentration (MCHC) is reported as 30%, how would this be interpreted in relation to the normal range?
Which formula correctly calculates the Mean Corpuscular Hemoglobin (MCH)?
Which formula correctly calculates the Mean Corpuscular Hemoglobin (MCH)?
What is the term used to describe a normal erythrocyte size, as defined by both diameter and MCV?
What is the term used to describe a normal erythrocyte size, as defined by both diameter and MCV?
What term describes the NORMAL variation in the color of erythrocytes, which you would report-out as "normochromic?"
What term describes the NORMAL variation in the color of erythrocytes, which you would report-out as "normochromic?"
What term is used when the central pallor of an erythrocyte exceeds one third of the cell's diameter?
What term is used when the central pallor of an erythrocyte exceeds one third of the cell's diameter?
Which condition is associated with microcytic hypochromic erythrocytes?
Which condition is associated with microcytic hypochromic erythrocytes?
When stained with Wright's stain, what color change is associated with erythrocyte polychromatophilia?
When stained with Wright's stain, what color change is associated with erythrocyte polychromatophilia?
Which cellular component is responsible for the blue color observed in polychromatophilic erythrocytes?
Which cellular component is responsible for the blue color observed in polychromatophilic erythrocytes?
What does the term 'normochromic' refer to in the context of erythrocyte color?
What does the term 'normochromic' refer to in the context of erythrocyte color?
In what type of cell would polychromatophilia be observed?
In what type of cell would polychromatophilia be observed?
What is indicated by the presence of polychromatophilia in a blood smear?
What is indicated by the presence of polychromatophilia in a blood smear?
What term describes the variation in SIZE observed in erythrocytes? For instance, when you could fit 1 or 2 small cells inside the outline of another, larger cell?
What term describes the variation in SIZE observed in erythrocytes? For instance, when you could fit 1 or 2 small cells inside the outline of another, larger cell?
Which of the following suffixes is used to denote alterations in the color of erythrocytes?
Which of the following suffixes is used to denote alterations in the color of erythrocytes?
What is the typical shape of a normal, mature erythrocyte (discocyte)?
What is the typical shape of a normal, mature erythrocyte (discocyte)?
Which term is used to describe the general variations observed in the shape of erythrocytes, but not enough of any one variation to report on its own?
Which term is used to describe the general variations observed in the shape of erythrocytes, but not enough of any one variation to report on its own?
Which of the following is TRUE regarding normal, mature erythrocytes?
Which of the following is TRUE regarding normal, mature erythrocytes?
Which of the following erythrocyte indices is used to express the average volume (size) of an erythrocyte?
Which of the following erythrocyte indices is used to express the average volume (size) of an erythrocyte?
What is indicated by performing an examination of erythrocyte distribution on a peripheral blood smear?
What is indicated by performing an examination of erythrocyte distribution on a peripheral blood smear?
Which of the following erythrocyte indices measures the concentration of hemoglobin within the cell?
Which of the following erythrocyte indices measures the concentration of hemoglobin within the cell?
Which of the following is NOT a type of Plasmodium that causes malaria?
Which of the following is NOT a type of Plasmodium that causes malaria?
What characteristic distinguishes Plasmodium vivax from other malaria-causing Plasmodium species?
What characteristic distinguishes Plasmodium vivax from other malaria-causing Plasmodium species?
Which of the following takes precedence in the categorization of Plasmodium species that cause malaria?
Which of the following takes precedence in the categorization of Plasmodium species that cause malaria?
Which malaria-like disease is associated with Babesia microti?
Which malaria-like disease is associated with Babesia microti?
What term describes mature erythrocytes that exhibit shapes other than the normal round, biconcave appearance?
What term describes mature erythrocytes that exhibit shapes other than the normal round, biconcave appearance?
Which of the following erythrocytes are classified as macrocytic?
Which of the following erythrocytes are classified as macrocytic?
Which variation in erythrocyte shape resembles a cell with pointy projections that are unevenly dispersed?
Which variation in erythrocyte shape resembles a cell with pointy projections that are unevenly dispersed?
What do schistocytes indicate about the erythrocyte's integrity?
What do schistocytes indicate about the erythrocyte's integrity?
Which characteristic is true of spherocytes?
Which characteristic is true of spherocytes?
Which erythrocyte shape is known for having rounded projections evenly dispersed around the cell?
Which erythrocyte shape is known for having rounded projections evenly dispersed around the cell?
What type of blood cell is classified as target cells?
What type of blood cell is classified as target cells?
What describes the appearance of keratocytes in a blood smear?
What describes the appearance of keratocytes in a blood smear?
Which type of erythrocytes can indicate a process of fragmentation?
Which type of erythrocytes can indicate a process of fragmentation?
What do polychromatic erythrocytes indicate when observed under a microscope? (note * marking near top-right)
What do polychromatic erythrocytes indicate when observed under a microscope? (note * marking near top-right)
Which cell morphology is characterized by a tear drop shape?
Which cell morphology is characterized by a tear drop shape?
Elliptocytes can be most accurately described as what shape?
Elliptocytes can be most accurately described as what shape?
In what condition would you likely observe an increased number of target cells?
In what condition would you likely observe an increased number of target cells?
What is the genetic requirement for an individual to express sickle cell anemia (Hb SS)?
What is the genetic requirement for an individual to express sickle cell anemia (Hb SS)?
Which of the following is the only potential cure for sickle cell anemia?
Which of the following is the only potential cure for sickle cell anemia?
Which nucleotide change in the DNA leads to the production of Hb S?
Which nucleotide change in the DNA leads to the production of Hb S?
Which medication, available over the counter, may help mitigate sickle formation by preventing water loss?
Which medication, available over the counter, may help mitigate sickle formation by preventing water loss?
What direct effect does Hb S have on red blood cells (RBCs) under deoxygenated conditions?
What direct effect does Hb S have on red blood cells (RBCs) under deoxygenated conditions?
What is the purpose of Splenectomy in Sickle Cell Disease?
What is the purpose of Splenectomy in Sickle Cell Disease?
What is the primary mechanism by which red blood cells affected by Hb S are removed from circulation?
What is the primary mechanism by which red blood cells affected by Hb S are removed from circulation?
The sickle cell carrier state provides a selective advantage against which disease?
The sickle cell carrier state provides a selective advantage against which disease?
Which of the following experimental treatments for sickle cell disease involves inserting a normal gene to replace the defective one?
Which of the following experimental treatments for sickle cell disease involves inserting a normal gene to replace the defective one?
What is the primary action attributed to nitric oxide in the context of managing vasoocclusive crises in sickle cell disease?
What is the primary action attributed to nitric oxide in the context of managing vasoocclusive crises in sickle cell disease?
Which factor does not promote the sickling of red blood cells?
Which factor does not promote the sickling of red blood cells?
Which of the following hemoglobins retards the sickling process?
Which of the following hemoglobins retards the sickling process?
In the US, newborns are screened for sickle cell anemia via heelstick onto filter paper. What is one of the two screening tests used most often?
In the US, newborns are screened for sickle cell anemia via heelstick onto filter paper. What is one of the two screening tests used most often?
What happens to the mean corpuscular hemoglobin concentration (MCHC) when sickling occurs?
What happens to the mean corpuscular hemoglobin concentration (MCHC) when sickling occurs?
What is an effect of decreased beta-globin production? (Decreased β-globin production = "β Thalassemia." So a decrease in β-globin must be compensated-for by an INCREASE in what?)
What is an effect of decreased beta-globin production? (Decreased β-globin production = "β Thalassemia." So a decrease in β-globin must be compensated-for by an INCREASE in what?)
Which of the following is a typical hematological finding in beta thalassemia?
Which of the following is a typical hematological finding in beta thalassemia?
What is observed on peripheral blood smears in patients with β thalassemia?
What is observed on peripheral blood smears in patients with β thalassemia?
Which of the following red cell indices is typically increased in β thalassemia?
Which of the following red cell indices is typically increased in β thalassemia?
Which laboratory finding is associated with β thalassemia?
Which laboratory finding is associated with β thalassemia?
Which hemoglobin is characteristically INCREASED in hemoglobin electrophoresis of a patient with β thalassemia?
Which hemoglobin is characteristically INCREASED in hemoglobin electrophoresis of a patient with β thalassemia?
What is a key characteristic of a particularly severe form of homozygous α thalassemia?
What is a key characteristic of a particularly severe form of homozygous α thalassemia?
Heterozygous β thalassemia can be mistaken for what condition on a peripheral blood smear?
Heterozygous β thalassemia can be mistaken for what condition on a peripheral blood smear?
Which of the following mutations is LEAST likely to be seen in thalassemia?
Which of the following mutations is LEAST likely to be seen in thalassemia?
Why do alpha thalassemias typically have higher severity than beta thalassemias?
Why do alpha thalassemias typically have higher severity than beta thalassemias?
In beta thalassemias, overall quantity of A1 globin is diminished along with beta globin. How is this lack of A1 and B globins compensated for?
In beta thalassemias, overall quantity of A1 globin is diminished along with beta globin. How is this lack of A1 and B globins compensated for?
What causes accelerated red cell destruction in alpha thalassemia?
What causes accelerated red cell destruction in alpha thalassemia?
Studies of RNA metabolism in erythroid cells have suggested that many patients with α thalassemia have a defect in what?
Studies of RNA metabolism in erythroid cells have suggested that many patients with α thalassemia have a defect in what?
What is a common outcome of mutations at the termination of a globin gene?
What is a common outcome of mutations at the termination of a globin gene?
Beta thalassemia is described by which of the following?
Beta thalassemia is described by which of the following?
What distinguishes a hemoglobinopathy disease from a hemoglobinopathy trait?
What distinguishes a hemoglobinopathy disease from a hemoglobinopathy trait?
Why are sickle cell disease (SCD) and beta-thalassemias prevalent in regions within the 'malaria belt'?
Why are sickle cell disease (SCD) and beta-thalassemias prevalent in regions within the 'malaria belt'?
In the context of hemoglobinopathies, what is a 'trait' specifically defined as?
In the context of hemoglobinopathies, what is a 'trait' specifically defined as?
Which genetic inheritance pattern must occur for sickle cell anemia to manifest as a disease?
Which genetic inheritance pattern must occur for sickle cell anemia to manifest as a disease?
What causes hemoglobinopathies like sickle cell disease?
What causes hemoglobinopathies like sickle cell disease?
Regarding hemoglobin defects, what determines the overall ZYGOSITY and quantity of hemoglobin produced?
Regarding hemoglobin defects, what determines the overall ZYGOSITY and quantity of hemoglobin produced?
In the context of hemoglobinopathies, what does the term 'qualitative protein changes' refer to?
In the context of hemoglobinopathies, what does the term 'qualitative protein changes' refer to?
What specific advantage do heterozygotes for hemoglobin mutations in the 'malaria belt' regions gain?
What specific advantage do heterozygotes for hemoglobin mutations in the 'malaria belt' regions gain?
What is the preferred method for prenatal diagnosis of β thalassemia during the first trimester?
What is the preferred method for prenatal diagnosis of β thalassemia during the first trimester?
Which technique is most commonly used in newborn screening programs for hemoglobinopathies?
Which technique is most commonly used in newborn screening programs for hemoglobinopathies?
What is the primary treatment method for severe anemia in patients with thalassemia?
What is the primary treatment method for severe anemia in patients with thalassemia?
What is a major cause of α thalassemia?
What is a major cause of α thalassemia?
In thalassemia, frequent blood transfusions can cause iron overload. What can be used to address the overload problem? (3 answers)
In thalassemia, frequent blood transfusions can cause iron overload. What can be used to address the overload problem? (3 answers)
How can genetic counseling and prenatal diagnosis impact the incidence of homozygous β thalassemia?
How can genetic counseling and prenatal diagnosis impact the incidence of homozygous β thalassemia?
In newborn screening for hemoglobinopathies, what additional information can guide the diagnostic process after an abnormal result?
In newborn screening for hemoglobinopathies, what additional information can guide the diagnostic process after an abnormal result?
What is a significant impact of frequent blood transfusions in thalassemia treatment?
What is a significant impact of frequent blood transfusions in thalassemia treatment?
What does a positive result in a solubility test indicate?
What does a positive result in a solubility test indicate?
If fetal hemoglobin is detected in an older person, what could this potentially indicate?
If fetal hemoglobin is detected in an older person, what could this potentially indicate?
An individual inherits hemoglobin E gene from one parent and S gene from the other. What condition does this result in?
An individual inherits hemoglobin E gene from one parent and S gene from the other. What condition does this result in?
Why might a specimen be sent to the CDC for hemoglobin analysis?
Why might a specimen be sent to the CDC for hemoglobin analysis?
What is the purpose of the Georgia General Assembly passing laws requiring sickle cell testing?
What is the purpose of the Georgia General Assembly passing laws requiring sickle cell testing?
In cellulose acetate electrophoresis at an alkaline pH, what determines the movement of hemoglobin molecules?
In cellulose acetate electrophoresis at an alkaline pH, what determines the movement of hemoglobin molecules?
During electrophoresis, if a hemoglobin molecule is placed in an ACID buffer solution, it will move towards which electrode?
During electrophoresis, if a hemoglobin molecule is placed in an ACID buffer solution, it will move towards which electrode?
In electrophoresis, assuming other factors are constant, which of the following hemoglobin types would migrate the furthest from the origin towards the anode?
In electrophoresis, assuming other factors are constant, which of the following hemoglobin types would migrate the furthest from the origin towards the anode?
Which component in a electrophoresis sample acts as a known reference point to compare patient hemoglobin types?
Which component in a electrophoresis sample acts as a known reference point to compare patient hemoglobin types?
What is the genetic composition of an asymptomatic sickle cell trait carrier?
What is the genetic composition of an asymptomatic sickle cell trait carrier?
How are hemoglobin molecules released from red blood cells (RBCs) prior to electrophoretic separation?
How are hemoglobin molecules released from red blood cells (RBCs) prior to electrophoretic separation?
Match the hemoglobin polypeptide chain combinations with their respective hemoglobin types:
Match the hemoglobin polypeptide chain combinations with their respective hemoglobin types:
Flashcards
Erythrocyte
Erythrocyte
A mature red blood cell with a biconcave shape, responsible for oxygen transport.
Erythropoiesis
Erythropoiesis
The process of producing red blood cells from stem cells.
Biconcave disc
Biconcave disc
Shape of mature erythrocytes that enhances gas exchange.
Hemoglobin
Hemoglobin
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Erythroblastic islands
Erythroblastic islands
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Cytoskeleton
Cytoskeleton
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Normoblasts
Normoblasts
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Spectrin
Spectrin
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Nutritional requirements
Nutritional requirements
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Aging RBC membrane
Aging RBC membrane
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Abnormal erythropoiesis
Abnormal erythropoiesis
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PMCA
PMCA
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Hemolysis
Hemolysis
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Polycythemia
Polycythemia
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Secondary Polycythemia
Secondary Polycythemia
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Relative Polycythemia
Relative Polycythemia
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Megaloblastic Maturation
Megaloblastic Maturation
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Vitamin B12 and Folate Deficiency
Vitamin B12 and Folate Deficiency
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Asynchronous Maturation
Asynchronous Maturation
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Hematocrit
Hematocrit
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Normal Reticulocyte Range (Adults)
Normal Reticulocyte Range (Adults)
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Normal Reticulocyte Range (Newborns)
Normal Reticulocyte Range (Newborns)
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Corrected Reticulocyte Count Formula
Corrected Reticulocyte Count Formula
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Reticulocyte Production Index (RPI)
Reticulocyte Production Index (RPI)
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RPI Calculation
RPI Calculation
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Mature Erythrocyte Characteristics
Mature Erythrocyte Characteristics
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Erythrocyte Size
Erythrocyte Size
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Erythrocyte Metabolism
Erythrocyte Metabolism
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Erythrocyte lifespan
Erythrocyte lifespan
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Anaerobic glycolysis
Anaerobic glycolysis
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Reduced Glutathione
Reduced Glutathione
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NADH
NADH
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Embden-Meyerhof pathway
Embden-Meyerhof pathway
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Methemoglobin reductase pathway
Methemoglobin reductase pathway
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G6PD Deficiency
G6PD Deficiency
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Rapoport-Luebering pathway
Rapoport-Luebering pathway
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Pyruvate Kinase
Pyruvate Kinase
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Embden-Meyerhof Pathway
Embden-Meyerhof Pathway
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Reticulocyte changes
Reticulocyte changes
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Erythrocyte membrane structure
Erythrocyte membrane structure
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Hexose Monophosphate Shunt
Hexose Monophosphate Shunt
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ATP and calcium in RBCs
ATP and calcium in RBCs
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Methemoglobin Reductase Pathway
Methemoglobin Reductase Pathway
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2,3-DPG
2,3-DPG
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2,3-Diphosphoglycerate (2,3-DPG)
2,3-Diphosphoglycerate (2,3-DPG)
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Oxyhemoglobin Dissociation Curve
Oxyhemoglobin Dissociation Curve
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P50 Value
P50 Value
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Hypoxia
Hypoxia
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Shifts of the Dissociation Curve
Shifts of the Dissociation Curve
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Direct Carbon Dioxide Transport
Direct Carbon Dioxide Transport
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Indirect Carbon Dioxide Transport
Indirect Carbon Dioxide Transport
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Carbonic Anhydrase
Carbonic Anhydrase
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Salt Bridges
Salt Bridges
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Role of Oxygen in Tissues
Role of Oxygen in Tissues
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Carbonic Acid Formation
Carbonic Acid Formation
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Chloride Shift
Chloride Shift
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Carbamino Hemoglobin
Carbamino Hemoglobin
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Bicarbonate Transport
Bicarbonate Transport
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Heme Production
Heme Production
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Iron's Role in Hemoglobin
Iron's Role in Hemoglobin
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Ferritin and Iron Storage
Ferritin and Iron Storage
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Globin Production
Globin Production
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Alpha Globin Locus
Alpha Globin Locus
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Beta Globin Locus
Beta Globin Locus
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Functional Hemoglobin
Functional Hemoglobin
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Porphyrias
Porphyrias
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Normal Hemoglobin Types
Normal Hemoglobin Types
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Sideroblastic Anemia
Sideroblastic Anemia
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Causes of Sideroblastic Anemia
Causes of Sideroblastic Anemia
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Hereditary Hemochromatosis
Hereditary Hemochromatosis
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Types of Hemochromatosis
Types of Hemochromatosis
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Globin Synthesis
Globin Synthesis
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Protoporphyrin Synthesis
Protoporphyrin Synthesis
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Defects in Globin Synthesis
Defects in Globin Synthesis
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Embryonic Hemoglobins
Embryonic Hemoglobins
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Molecular Testing
Molecular Testing
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Thalassemias
Thalassemias
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Erythrocyte Aging
Erythrocyte Aging
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Extravascular Catabolism
Extravascular Catabolism
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Components from Erythrocyte Breakdown
Components from Erythrocyte Breakdown
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Transferrin
Transferrin
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Intravascular Catabolism
Intravascular Catabolism
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Haptoglobin
Haptoglobin
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Sulfhemoglobin
Sulfhemoglobin
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Methemoglobin
Methemoglobin
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Cyanosis
Cyanosis
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Sickle Cell Anemia
Sickle Cell Anemia
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Heinz Bodies
Heinz Bodies
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Electrophoresis
Electrophoresis
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Alkaline Electrophoresis
Alkaline Electrophoresis
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Homozygous vs Heterozygous
Homozygous vs Heterozygous
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Mean Corpuscular Volume (MCV)
Mean Corpuscular Volume (MCV)
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Mean Corpuscular Hemoglobin (MCH)
Mean Corpuscular Hemoglobin (MCH)
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Mean Corpuscular Hemoglobin Concentration (MCHC)
Mean Corpuscular Hemoglobin Concentration (MCHC)
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Normocytic
Normocytic
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Erythrocyte Size Variation
Erythrocyte Size Variation
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Reticulocytes
Reticulocytes
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Basophilic Stippling
Basophilic Stippling
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Howell-Jolly Bodies
Howell-Jolly Bodies
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Pappenheimer Bodies
Pappenheimer Bodies
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Plasmodium vivax
Plasmodium vivax
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Plasmodium falciparum
Plasmodium falciparum
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Plasmodium malariae
Plasmodium malariae
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Plasmodium ovale
Plasmodium ovale
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Leishmania spp.
Leishmania spp.
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Central Pallor
Central Pallor
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Normochromic
Normochromic
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Anisochromia
Anisochromia
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Hypochromia
Hypochromia
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Polychromatophilia
Polychromatophilia
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Iron Deficiency Anemia
Iron Deficiency Anemia
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Cell Immaturity Indicator
Cell Immaturity Indicator
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Anisocytosis
Anisocytosis
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Poikilocytosis
Poikilocytosis
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Normal mature erythrocytes
Normal mature erythrocytes
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Erythrocyte Inclusions
Erythrocyte Inclusions
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Alteration in color (-chromia)
Alteration in color (-chromia)
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Macrocytic Erythrocytes
Macrocytic Erythrocytes
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Microcytic Erythrocytes
Microcytic Erythrocytes
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Acanthocytes
Acanthocytes
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Blister Cells
Blister Cells
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Keratocytes
Keratocytes
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Schistocytes
Schistocytes
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Echinocytes
Echinocytes
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Elliptocytes
Elliptocytes
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Ovalocytes
Ovalocytes
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Spherocytes
Spherocytes
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Dacryocytes
Dacryocytes
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Codocytes
Codocytes
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Polychromasia
Polychromasia
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Hemoglobinopathies
Hemoglobinopathies
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Sickle Cell Disease (SCD)
Sickle Cell Disease (SCD)
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Heterozygous Trait
Heterozygous Trait
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Malaria Belt
Malaria Belt
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Hemoglobin S (Hb S)
Hemoglobin S (Hb S)
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Etiology of Sickle Cell Anemia
Etiology of Sickle Cell Anemia
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Polymerization of Hb S
Polymerization of Hb S
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Sickle Cell Carrier Advantage
Sickle Cell Carrier Advantage
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Pathophysiology of Sickling
Pathophysiology of Sickling
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Extramedullary Hematopoiesis
Extramedullary Hematopoiesis
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Nonsense Mutation
Nonsense Mutation
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Thalassemia Types
Thalassemia Types
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Alpha Thalassemia Impact
Alpha Thalassemia Impact
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Beta Thalassemia Compensation
Beta Thalassemia Compensation
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Inefficient RNA Splicing
Inefficient RNA Splicing
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Globin Gene Depletion
Globin Gene Depletion
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Hematopoiesis
Hematopoiesis
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Mendelian Genetics
Mendelian Genetics
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Initial Screening Techniques
Initial Screening Techniques
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Sickle Cell Disease Management
Sickle Cell Disease Management
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Bone Marrow Transplant
Bone Marrow Transplant
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Gene Therapy
Gene Therapy
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Preventive Vaccinations
Preventive Vaccinations
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Clotrimazole
Clotrimazole
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Splenectomy
Splenectomy
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Butyric Acid
Butyric Acid
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β Thalassemia
β Thalassemia
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Ineffective Erythropoiesis
Ineffective Erythropoiesis
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Laboratory Findings of β Thalassemia
Laboratory Findings of β Thalassemia
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Hemoglobin Electrophoresis
Hemoglobin Electrophoresis
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Increased Hb F
Increased Hb F
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Reticulocyte Formation
Reticulocyte Formation
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Soluble Transferrin Receptor Index
Soluble Transferrin Receptor Index
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Prenatal Diagnosis of β Thalassemia
Prenatal Diagnosis of β Thalassemia
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Newborn Screening Techniques
Newborn Screening Techniques
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Blood Transfusion in Anemia
Blood Transfusion in Anemia
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Iron Overload Treatment
Iron Overload Treatment
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Alpha Thalassemia Causes
Alpha Thalassemia Causes
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Beta-to-Alpha Ratio
Beta-to-Alpha Ratio
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Prenatal Counseling Impact
Prenatal Counseling Impact
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Genetic Testing for Thalassemia
Genetic Testing for Thalassemia
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Sickle Cell Disease
Sickle Cell Disease
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Fetal Hemoglobin
Fetal Hemoglobin
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Hemoglobin Interaction
Hemoglobin Interaction
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Solubility Test
Solubility Test
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Electrophoretic Separation
Electrophoretic Separation
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Hemoglobin Types
Hemoglobin Types
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Marriage License Testing
Marriage License Testing
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Centrifugation Steps
Centrifugation Steps
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Hemoglobin Charge at pH
Hemoglobin Charge at pH
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Inheritance of Hemoglobin Genes
Inheritance of Hemoglobin Genes
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Control Sample in Electrophoresis
Control Sample in Electrophoresis
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Study Notes
Erythrocytes: Overview
- Erythrocytes (red blood cells) are biconcave discs, with a central pallor.
- Hemoglobin transports oxygen and carbon dioxide.
- Mature red blood cells circulate for an average of 120 days.
- Membrane rigidity increases with age, leading to phagocytosis and destruction.
Erythropoiesis
- Erythropoiesis is the production of red blood cells.
- It originates from hematopoietic stem cells (HSCs).
- Erythropoiesis occurs in erythroblastic islands, clusters of normoblasts (erythroblasts) around iron-laden macrophages.
- These macrophages provide iron and cytokines necessary for hemoglobin maturation and erythroid progenitor cell maturation.
- Essential components include amino acids, iron, vitamin B12, vitamin B6, folic acid, and trace minerals (cobalt and nickel).
- Abnormal erythropoiesis can arise from deficiencies in these substances.
Erythropoietin (EPO)
- EPO is primarily produced by the peritubular cells of the kidneys (80-90%).
- The remaining 10-20% is produced in the liver (especially in fetal development).
- Blood EPO levels are inversely proportional to tissue oxygenation.
- EPO affects early and late-acting cytokines on erythroid progenitors, promoting their maturation and differentiation.
- EPO also interacts with other cytokines (IL-3, GM-CSF, IL-1, and TSF) for maturation and differentiation purposes.
- Erythropoietin (EPO) is crucial for the rate of red blood cell production.
Maturation and Development
- Erythrocyte maturation takes approximately 4-5 days, proceeding through nucleated stages.
- Bone marrow reticulocytes mature for an average of 2.5 days.
- Circulating reticulocytes have a lifespan of about 1 day.
- Reticulocytes represent roughly 0.5% - 1.5% of circulating erythrocytes.
Cell Morphology (Rubriblasts, Prorubricytes, Rubricytes, Metarubricytes)
- Detailed descriptions of the morphology (size, nuclear characteristics, cytoplasmic features) of each stage of erythroid maturation are provided in the images. These include characteristics like nuclear size, chromatin pattern, presence of nucleoli, and cytoplasmic color/staining, enabling identification of each stage.
Reticulocytes
- Reticulocytes are immature red blood cells.
- A supravital stain like new methylene blue can be used to identify residual RNA as a mesh-like network.
- Reticulocyte counts assess red blood cell production rate.
Reticulocyte Production Index (RPI)
- Uses the corrected reticulocyte percentage and maturation time to determine effective erythropoiesis. RPI corrects for the presence of abnormal or prematurely released reticulocytes. Various hematocrit levels have associated correction factors for this calculation. RPI is a more informative measure of bone marrow function than reticulocyte percentage alone.
Mature Erythrocyte
- Mature red blood cells are anucleate.
- They transport oxygen and carbon dioxide via hemoglobin.
- They have an average lifespan of 120 days. Their function involves oxygen and carbon dioxide transport. They lack organelles and perform crucial functions without a nucleus. Their biconcave shape and flexible membrane are critical for efficient gas exchange.
Metabolic Activities
- Embden-Meyerhof-Parnas pathway is the primary anaerobic glycolysis pathway producing ATP. This is essential for the erythrocyte's metabolic needs.
- Hexose monophosphate shunt provides reducing equivalents and protects from oxidative damage, crucial for preventing cellular damage.
- Methemoglobin reductase pathway maintains hemoglobin in its reduced state, necessary for optimal oxygen transport.
- Luebering-Rapoport pathway maintains 2,3-DPG levels, and supports oxygen delivery to the tissues, an important aspect of transporting oxygen.
Membrane Characteristics
- Red blood cell membranes have a complex protein skeleton connected to the lipid bilayer, enabling their deformability and flexibility. These proteins, like spectrins, ankyrins, and glycophorins, maintain the biconcave shape and flexibility needed for microcirculation and function in capillaries.
- Age-related changes occur, including alterations in surface area, lipid components, and membrane protein function. Membrane protein integrity and interactions are crucial for function. Different proteins play distinct structural and functional roles.
- Characteristics change with age and are influenced by several factors (e.g., ATP levels, intracellular calcium), which directly affect the cell's deformability and lifespan.
Disorders of Erythropoiesis
- Polycythemia is an increase in red blood cell count, which can be primary (polycythemia vera) or secondary (e.g., high altitude, chronic lung disease).
- Anemia can result from decreased red blood cell production or increased destruction/loss, and can have many causes.
Defective Nuclear Maturation
- Issues with DNA synthesis can cause defects in nuclear maturation, leading to megaloblastic red blood cell production in conditions like vitamin B12 or folate deficiency.
- Cytoplasmic maturation often occurs faster than nuclear development in these cases, leading to characteristic morphological abnormalities.
Additional Considerations
- Powerpoint slides are summaries, and full details are found in the textbook.
- Images and descriptions of cell structure and function are included as summaries.
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