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Erythrocytes Structure and Function
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Erythrocytes Structure and Function

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Questions and Answers

What is the normal life span of erythrocytes?

  • 90 days
  • 120 days (correct)
  • 150 days
  • 60 days

    • What is the approximate diameter range of erythrocytes?

  • 12-14 um
  • 6-8 um (correct)
  • 3-5 um
  • 9-11 um

    • Growth factor that stimulates erythrocyte production from myeloid progenitor cells and influences colony-forming unit-erythrocytes (CFU-Es) to differentiate into erythroblasts?

  • Erythropoietin (correct)
  • Insulin-like growth factor 1
  • Interleukin-3
  • Tumor necrosis factor

    • What is the central pallor size of erythrocytes?

    <p>Less than one-third</p> Signup and view all the answers

    What is the diameter of erythrocytes, on average, in comparison to the size of the nucleus of a small lymphocyte?

    <p>Approximately the same</p> Signup and view all the answers

    Which type of progenitor cell does erythropoietin stimulate to produce erythrocytes?

    <p>Myeloid progenitor cell</p> Signup and view all the answers

    Where is erythropoietin mainly produced?

    <p>Kidneys</p> Signup and view all the answers

    What condition is associated with increased central pallor of erythrocytes?

    <p>Microcytic anemias</p> Signup and view all the answers

    What regulates erythropoiesis in addition to erythropoietin?

    <p>Androgens</p> Signup and view all the answers

    What is the reference range of red blood cell count for males in conventional units?

    <p>4.6-6.0 X 106/uL</p> Signup and view all the answers

    What role does androgen play in erythropoiesis?

    <p>Enhances the activity of erythropoietin</p> Signup and view all the answers

    What condition leads to a decrease in central pallor of erythrocytes?

    <p>Both</p> Signup and view all the answers

    Hemoglobin continues to be produced by reticulocytes for approximately ___ hours after exiting the bone marrow?

    <p>~24 hours</p> Signup and view all the answers

    What is the size range of a polychromatophilic normoblast (rubricyte)?

    <p>Up to 12 μm</p> Signup and view all the answers

    Which normoblast has an eccentric nucleus with small, fully condensed (pyknotic) nucleus and pale blue to salmon cytoplasm?

    <p>Orthochromic normoblast (metarubricyte)</p> Signup and view all the answers

    Which normoblast begins to produce hemoglobin, resulting in gray-blue cytoplasm?

    <p>Polychromatophilic normoblast (rubricyte)</p> Signup and view all the answers

    What is the N:C ratio of a pronormoblast (rubriblast)?

    <p>8:1</p> Signup and view all the answers

    Which type of cell contains no nucleus but has mitochondria and ribosomes?

    <p>Reticulocyte</p> Signup and view all the answers

    What is the approximate size of an orthochromic normoblast (metarubricyte)?

    <p>Up to 10 μm</p> Signup and view all the answers

    What is the primary source of energy for early red blood cells?

    <p>Oxidative phosphorylation</p> Signup and view all the answers

    What is the best indicator of bone marrow function?

    <p>Reticulocyte count</p> Signup and view all the answers

    Which mechanism is responsible for maintaining the proper volume ratio and flexibility of erythrocytes?

    <p>Cation pump</p> Signup and view all the answers

    What is the approximate lipid composition of the erythrocyte membrane?

    <p>50-60% lipid</p> Signup and view all the answers

    In which type of anemia are macrocytes (RBCs > 8 um in diameter) commonly seen?

    <p>Megaloblastic anemias</p> Signup and view all the answers

    What is the size range of microcytes?

    <p>&lt; 6 fL</p> Signup and view all the answers

    Which substance is required for the transport of oxygen in erythropoiesis?

    <p>Iron in the ferrous state (Fe2+)</p> Signup and view all the answers

    In which type of anemia are microcytes (RBCs less than 6 um in diameter) commonly seen?

    <p>AOTA</p> Signup and view all the answers

    What is the defining characteristic of normocytes (discocytes)?

    <p>Approximately the same size as the nucleus of a small lymphocyte</p> Signup and view all the answers

    What does anisocytosis indicate in a red blood cell population?

    <p>Variation in RBC size</p> Signup and view all the answers

    In which condition is an increase in RDW (red blood cell distribution width) most likely to be seen?

    <p>Post-transfusion</p> Signup and view all the answers

    When is dimorphism, a heterogeneous RBC population, most likely to be observed?

    <p>Presence of two concurrent deficiencies (e.g., iron and vitamin B12)</p> Signup and view all the answers

    What is the general term used to describe variation in shape of red blood cells?

    <p>Poikilocytosis</p> Signup and view all the answers

    Which condition is associated with evenly spaced sharp projections and a central pallor area in red blood cells?

    <p>Liver disease</p> Signup and view all the answers

    What condition is commonly associated with unevenly spiculated red blood cells with bulbous and rounded ends?

    <p>Alcoholic liver disease</p> Signup and view all the answers

    Which condition is associated with red blood cells showing a central area of hemoglobin surrounded by a colorless ring and a peripheral ring of hemoglobin?

    <p>Hemoglobinopathies</p> Signup and view all the answers

    In which conditions are poikilocytosis observed?

    <p>Liver disease and uremia</p> Signup and view all the answers

    What is the distinguishing characteristic of acanthocytes?

    <p>Unevenly spiculated cells with bulbous and rounded ends</p> Signup and view all the answers

    What is the defining characteristic of acanthocytes?

    <p>Unevenly spiculated cells with bulbous and rounded ends</p> Signup and view all the answers

    What causes the formation of echinocytes (crenated and burr cells)?

    <p>Changes in osmotic pressure</p> Signup and view all the answers

    What is the cause of spiculated red blood cells with an increased surface-to-volume ratio?

    <p>Excessive cholesterol in the membrane</p> Signup and view all the answers

    What causes the excessive cholesterol in the membrane of crenated cells?

    <p>Post-splenectomy</p> Signup and view all the answers

    What condition is commonly associated with target cells (codocytes or Mexican hat cells)?

    <p>Hemoglobinopathies</p> Signup and view all the answers

    Which condition is associated with evenly spaced sharp projections and a central pallor area in red blood cells?

    <p>Liver disease</p> Signup and view all the answers

    Which type of red blood cell is associated with defects of the red cell membrane proteins?

    <p>Elliptocytes</p> Signup and view all the answers

    In which condition are sickle cells most commonly seen?

    <p>Hemoglobinopathies SS</p> Signup and view all the answers

    What causes the cell shape of schistocytes (RBC fragments) in microangiopathic hemolytic anemias?

    <p>Damage to RBCs</p> Signup and view all the answers

    What is the defining characteristic of schistocytes (RBC fragments)?

    <p>Pointed projections</p> Signup and view all the answers

    Which condition is associated with pear-shaped cells and one blunt projection?

    <p>Megaloblastic anemias</p> Signup and view all the answers

    What is the characteristic feature of spherocytes?

    <p>Smaller volume than a normal erythrocyte</p> Signup and view all the answers

    What causes the characteristic cell shape of sickle cells?

    <p>Polymers of abnormal hemoglobin S</p> Signup and view all the answers

    What is the defining characteristic of stomatocytes (mouth cells)?

    <p>Elongated or slit-like area of central pallor</p> Signup and view all the answers

    Which condition is most commonly associated with the presence of teardrop cells (dacryocytes) in the blood?

    <p>Megaloblastic anemia</p> Signup and view all the answers

    What is the characteristic cell shape of spherocytes associated with?

    <p>Defects of red cell membrane proteins</p> Signup and view all the answers

    Which condition is commonly associated with the presence of schistocytes (RBC fragments) in the blood?

    <p>Hemolytic anemia</p> Signup and view all the answers

    What is the most likely cause of stomatocytes in the blood?

    <p>Cation imbalance (Na+/K+)</p> Signup and view all the answers

    What condition is associated with the presence of elliptocytes (ovalocytes) in the blood?

    <p>Hereditary stomatocytosis</p> Signup and view all the answers

    In which type of anemia are pencil forms of elliptocytes most commonly seen?

    <p>Iron-deficiency anemia</p> Signup and view all the answers

    Which of the following diseases is NOT associated with an increased presence of nucleated RBCs in the peripheral blood smear?

    <p>Iron-deficiency anemia</p> Signup and view all the answers

    In which condition would a few nucleated RBCs in a peripheral blood smear be considered normal?

    <p>Newborns</p> Signup and view all the answers

    Which disease is NOT commonly associated with the presence of nucleated RBCs in the peripheral blood smear?

    <p>Iron-deficiency anemia</p> Signup and view all the answers

    Which condition is most likely to be associated with the presence of nucleated RBCs in the peripheral blood smear?

    <p>Thalassemia major</p> Signup and view all the answers

    What is the significance of the presence of orthochromic normoblasts (metarubricytes) in the peripheral blood smear?

    <p>Suggests healthy erythropoiesis</p> Signup and view all the answers

    In which of the following disorders would a normal newborn most likely have a few nucleated RBCs on a peripheral blood smear?

    <p>Normal newborns should have none</p> Signup and view all the answers

    Which condition is NOT commonly associated with the presence of Howell-Jolly bodies in the blood?

    <p>Hereditary spherocytosis</p> Signup and view all the answers

    What is the most likely cause of Howell-Jolly bodies in erythrocytes?

    <p>Pitting by splenic macrophages</p> Signup and view all the answers

    In which condition are Howell-Jolly bodies most commonly seen?

    <p>Post-splenectomy</p> Signup and view all the answers

    Which condition is likely to show the 'blueberry bagel' appearance in red blood cells?

    <p>Lead poisoning</p> Signup and view all the answers

    What is the characteristic appearance of basophilic stippling in red blood cells?

    <p>Dark blue speckles</p> Signup and view all the answers

    Which of the following conditions is associated with basophilic stippling in red blood cells?

    <p>Sideroblastic anemia</p> Signup and view all the answers

    What is the characteristic staining pattern of Pappenheimer bodies with Wright's stain?

    <p>Dark violet</p> Signup and view all the answers

    What accumulates to cause the formation of Pappenheimer bodies?

    <p>Mitochondria and ribosomes</p> Signup and view all the answers

    In which of the following conditions are Pappenheimer bodies commonly observed?

    <p>Sideroblastic anemia</p> Signup and view all the answers

    What is the significance of Cabot rings in the peripheral blood smear?

    <p>They may be associated with megaloblastic anemia or myelodysplastic syndromes</p> Signup and view all the answers

    Which of the following correctly describes the composition of Cabot rings?

    <p>They are made up of fragments of nuclear material</p> Signup and view all the answers

    In which type of anemia is the presence of Cabot rings most commonly associated?

    <p>Megaloblastic anemia</p> Signup and view all the answers

    What type of crystal is condensed and rod-shaped?

    <p>Hemoglobin C crystals</p> Signup and view all the answers

    What is the defining characteristic of Hemoglobin SC crystals?

    <p>1-2 blunt, fingerlike projections extending from the cell membrane</p> Signup and view all the answers

    In which condition are HEMOGLOBIN SC CRYSTALS (WASHINGTON MONUMENT) observed?

    <p>Hemoglobin SC disease</p> Signup and view all the answers

    Which condition is most likely to be associated with the presence of Heinz bodies in red blood cells?

    <p>G6PD deficiency</p> Signup and view all the answers

    Which of the following is a condition where Heinz bodies may be seen due to denatured hemoglobin?

    <p>Beta-thalassemia major</p> Signup and view all the answers

    In which of the following conditions might Heinz bodies be visualized using a supravital stain?

    <p>Malaria infection</p> Signup and view all the answers

    What is the distinguishing characteristic of normochromasia?

    <p>Normal one-third clear, central pallor area</p> Signup and view all the answers

    What condition is often associated with hypochromasia?

    <p>Iron-deficiency anemia</p> Signup and view all the answers

    In which type of anemia are microcytosis and hypochromasia commonly seen?

    <p>Iron-deficiency anemia</p> Signup and view all the answers

    Qual es le principal characteristicas de polychromasia in le hemoglobina?

    <p>Spectaculo un tenuitate blau in le coloration con le colorante de Wright</p> Signup and view all the answers

    Qual es le causa plus probabile del macrocytosis observate con polychromasia?

    <p>Deficiente de vitamina B12 o acido folico</p> Signup and view all the answers

    Qual es le signification de un tintura blau que se observa in le hemoglobina quando colorate con Wright?

    <p>Mostra un diminution in le quantitate de RNA residual</p> Signup and view all the answers

    What is the definitive method for determining true rouleaux formation?

    <p>Determining in the thin area of the peripheral smear</p> Signup and view all the answers

    In which conditions would one most likely see excessively blue color to the smear macroscopically and microscopically?

    <p>Hyperproteinemia and multiple myeloma</p> Signup and view all the answers

    What causes the stacking or 'coining' pattern of erythrocytes known as rouleaux formation?

    <p>Increased plasma proteins</p> Signup and view all the answers

    What is the characteristic feature of agglutination in cold autoimmune hemolytic anemia?

    <p>Clumping of erythrocytes without any pattern</p> Signup and view all the answers

    What is the corrective measure for a false low RBC and hematocrit, and false high MCHC (>37 g/dL) when using an automated cell counting instrument in cold agglutinin disease?

    <p>Warming the blood to 37°C</p> Signup and view all the answers

    Which type of antibodies and complement lead to agglutination of erythrocytes in cold autoimmune hemolytic anemia?

    <p>IgM antibodies and complement</p> Signup and view all the answers

    What is the primary function of hemoglobin?

    <p>Transporting oxygen</p> Signup and view all the answers

    Which amino acid chain determines the type of hemoglobin?

    <p>Beta, gamma, and delta chains with 146 amino acids</p> Signup and view all the answers

    What gives erythrocytes their characteristic red color?

    <p>Ferrous (Fe2+) iron</p> Signup and view all the answers

    What is the role of the amino acid sequence in the globin chain of hemoglobin?

    <p>Determining the type of hemoglobin</p> Signup and view all the answers

    What gives erythrocytes their characteristic red color?

    <p>Heme portion of hemoglobin</p> Signup and view all the answers

    How many heme groups are present in the structure of hemoglobin?

    <p>Four</p> Signup and view all the answers

    Where does heme synthesis occur in the immature red blood cell?

    <p>Mitochondria</p> Signup and view all the answers

    Which chromosome controls the globin synthesis for alpha chains?

    <p>Chromosome 16</p> Signup and view all the answers

    What is the primary determinant of globin synthesis location?

    <p>Ribosomes</p> Signup and view all the answers

    Where does heme synthesis occur in the immature red blood cell?

    <p>Mitochondria</p> Signup and view all the answers

    What is the primary source of energy for early red blood cells?

    <p>Glucose</p> Signup and view all the answers

    Which amino acid chain determines the type of hemoglobin?

    <p>Globin</p> Signup and view all the answers

    What is the major free hemoglobin transport protein that binds to released free hemoglobin in intravascular hemolysis?

    <p>Haptoglobin</p> Signup and view all the answers

    Which laboratory parameter is most likely to be decreased in the context of intravascular hemolysis?

    <p>Serum haptoglobin</p> Signup and view all the answers

    What is the likely result in urine associated with intravascular hemolysis?

    <p>Presence of hemosiderin</p> Signup and view all the answers

    Where does the protoporphyrin ring get metabolized to bilirubin and urobilinogen in extravascular hemolysis?

    <p>Liver</p> Signup and view all the answers

    What happens to the globin chains of senescent/old RBCs during extravascular hemolysis?

    <p>Recycled into the amino acid pool for protein synthesis</p> Signup and view all the answers

    What happens to the iron in senescent/old RBCs during extravascular hemolysis?

    <p>Binds to transferrin and is transported to bone marrow for new RBC production</p> Signup and view all the answers

    What does serum ferritin measure?

    <p>The indirect measurement of storage iron in tissues and bone marrow</p> Signup and view all the answers

    What is the primary role of ferric iron (Fe3+) in the body?

    <p>Binding to transferrin</p> Signup and view all the answers

    What is the purpose of measuring total iron-binding capacity (TIBC)?

    <p>To determine the total amount of iron that transferrin can bind when fully saturated</p> Signup and view all the answers

    Which iron state is essential for binding to hemoglobin for transport to lungs and body tissues?

    <p>Ferrous state (Fe2+)</p> Signup and view all the answers

    What does serum ferritin measure indirectly in the body?

    <p>Storage iron in tissues and bone marrow</p> Signup and view all the answers

    What does total iron-binding capacity (TIBC) measure in the body?

    <p>Total amount of iron that transferrin can bind when fully saturated</p> Signup and view all the answers

    Which type of hemoglobin predominates at birth?

    <p>Hgb F</p> Signup and view all the answers

    Which laboratory test can be used to identify Hgb F due to its resistance to denaturation/elution?

    <p>Alkali denaturation test</p> Signup and view all the answers

    In which conditions can Hgb F be increased as a compensatory hemoglobin?

    <p>Thalassemia major</p> Signup and view all the answers

    What is the primary function of Hemoglobin Hgb F?

    <p>Functioning in a reduced oxygen environment</p> Signup and view all the answers

    At what age is gamma chain production complete, and beta chain production takes over?

    <p>6 months</p> Signup and view all the answers

    Which laboratory test can be used to identify Hemoglobin Hgb F due to its resistance to denaturation/elution?

    <p>Column chromatography</p> Signup and view all the answers

    What type of hemoglobin has 200 times more affinity for carbon monoxide than for oxygen?

    <p>Carboxyhemoglobin</p> Signup and view all the answers

    In which circulation is deoxyhemoglobin primarily seen?

    <p>Venous circulation</p> Signup and view all the answers

    What is the most likely outcome of a person who inhales carbon monoxide (CO)?

    <p>Death due to the affinity of CO for hemoglobin</p> Signup and view all the answers

    In which type of circulation is deoxyhemoglobin primarily seen?

    <p>Venous circulation</p> Signup and view all the answers

    What happens to the iron in deoxyhemoglobin?

    <p>It remains in the ferrous state but without oxygen</p> Signup and view all the answers

    Which type of hemoglobin cannot transport O2 and is caused by drugs and chemicals?

    <p>Sulfhemoglobin</p> Signup and view all the answers

    What is the effect of increased levels of methemoglobin?

    <p>Cyanosis and anemia</p> Signup and view all the answers

    Which type of hemoglobin has Fe3+ and cannot transport O2?

    <p>Methemoglobin</p> Signup and view all the answers

    What is the distinguishing characteristic of Sulfhemoglobin?

    <p>Measured by the cyanmethemoglobin method</p> Signup and view all the answers

    What is the effect of increased levels of Methemoglobin?

    <p>Decreased oxygen transport and cyanosis</p> Signup and view all the answers

    Which method is used to measure Sulfhemoglobin levels?

    <p>Spectrophotometry</p> Signup and view all the answers

    What does the term 'oxygen affinity' refer to in the context of hemoglobin?

    <p>The expression of hemoglobin in terms of oxygen tension</p> Signup and view all the answers

    What is the significance of hemoglobin being 50% saturated with oxygen?

    <p>It reflects a balance between oxygen binding and release by hemoglobin</p> Signup and view all the answers

    In the context of oxygen transport, what does '50% saturated with oxygen' mean for hemoglobin?

    <p>Hemoglobin is partially saturated with oxygen</p> Signup and view all the answers

    What effect does a high level of 2,3-bisphosphoglycerate (2,3-BPG) have on the oxygen affinity of hemoglobin?

    <p>It decreases oxygen affinity</p> Signup and view all the answers

    What effect does increased body temperature have on the oxygen affinity of hemoglobin?

    <p>It decreases oxygen affinity</p> Signup and view all the answers

    How does a left shift in the oxygen dissociation curve affect the release of oxygen to the tissues?

    <p>Less oxygen is released to the tissues</p> Signup and view all the answers

    What effect does a high level of 2,3-bisphosphoglycerate (2,3-BPG) have on the oxygen affinity of hemoglobin?

    <p>Decreases oxygen affinity</p> Signup and view all the answers

    What is the impact of increased body temperature on hemoglobin's oxygen affinity?

    <p>Increases oxygen affinity</p> Signup and view all the answers

    What is the consequence of decreased body pH on hemoglobin's oxygen affinity?

    <p>Decreases oxygen affinity</p> Signup and view all the answers

    In which condition would the mean corpuscular volume (MCV) most likely be increased?

    <p>Megaloblastic anemia</p> Signup and view all the answers

    Which of the following conditions is NOT associated with an increased mean corpuscular volume (MCV)?

    <p>Sideroblastic anemia</p> Signup and view all the answers

    What condition is most likely to cause a decreased mean corpuscular volume (MCV)?

    <p>Sideroblastic anemia</p> Signup and view all the answers

    What is the expected impact on mean corpuscular volume (MCV) in a patient with lead poisoning?

    <p>Decreased MCV</p> Signup and view all the answers

    What would be the expected impact on mean corpuscular volume (MCV) if the hematocrit (Hct) increases significantly?

    <p>Decrease</p> Signup and view all the answers

    If the RBC count decreases, what effect would it have on the mean corpuscular volume (MCV)?

    <p>Increase</p> Signup and view all the answers

    How would an increase in both hematocrit (Hct) and RBC count affect the mean corpuscular volume (MCV)?

    <p>Remain unchanged</p> Signup and view all the answers

    What is the formula for calculating Mean Corpuscular Hemoglobin (MCH)?

    <p>MCH (pg) = Hemoglobin (g/dL) X 10 / RBC count (X 10^12/L)</p> Signup and view all the answers

    In which type of anemia is Mean Corpuscular Hemoglobin (MCH) commonly increased?

    <p>Macrocytic anemia</p> Signup and view all the answers

    What change in the Mean Corpuscular Hemoglobin (MCH) is expected in microcytic, hypochromic anemia?

    <p>Decreased MCH</p> Signup and view all the answers

    What does the MCHC (Mean Corpuscular Hemoglobin Concentration) measure?

    <p>The average concentration of hemoglobin in red blood cells</p> Signup and view all the answers

    What is the significance of MCHC being lower than the reference range?

    <p>It may indicate hypochromic red blood cells with reduced hemoglobin content</p> Signup and view all the answers

    What does a Mean Corpuscular Hemoglobin Concentration (MCHC) of lesser than 32 g/dL indicate?

    <p>Possible error in RBC or hemoglobin measurement</p> Signup and view all the answers

    What is the significance of MCHC being within the range of 32-37 g/dL?

    <p>Normochromic RBCs</p> Signup and view all the answers

    When MCHC is greater than 37 g/dL, what could it indicate?

    <p>Presence of spherocytes</p> Signup and view all the answers

    What is the main morphological classification used to classify anemia?

    <p>RBC indices (MCV, MCH, and MCHC)</p> Signup and view all the answers

    In anemia, the decreased oxygen delivery to the tissues is primarily caused by a decrease in:

    <p>Hemoglobin concentration</p> Signup and view all the answers

    Anemia can also be classified based on its etiology/cause. Which of the following is a common cause of anemia?

    <p>Decreased hemoglobin levels</p> Signup and view all the answers

    What is the likely cause of relative anemia as described in the text?

    <p>Volume overload</p> Signup and view all the answers

    In the context of relative anemia, what would be the expected impact on the reticulocyte count?

    <p>Normal reticulocyte count</p> Signup and view all the answers

    What type of anemia is described in the text based on the characteristics provided?

    <p>Normocytic, normochromic anemia</p> Signup and view all the answers

    What is the mechanism involved in increased loss of red cells from the circulation?

    <p>Accelerated destruction and bone marrow response</p> Signup and view all the answers

    What is indicative of a true decrease in erythrocytes and hemoglobin?

    <p>Reticulocytopenia</p> Signup and view all the answers

    What does absolute anemia indicate?

    <p>Decreased RBC mass but normal plasma volume</p> Signup and view all the answers

    In which group of individuals is iron-deficiency anemia most prevalent?

    <p>Individuals with malabsorption syndromes</p> Signup and view all the answers

    What is the primary cause of iron-deficiency anemia in individuals with chronic blood loss?

    <p>Hookworm infection</p> Signup and view all the answers

    Which physiological event commonly contributes to iron-deficiency anemia in women?

    <p>Pregnancy</p> Signup and view all the answers

    What is the characteristic appearance of the smear in iron-deficiency anemia?

    <p>Ovalocytes/pencil forms and hypochromia/microcytosis</p> Signup and view all the answers

    Which clinical symptom is commonly associated with iron-deficiency anemia?

    <p>Pica</p> Signup and view all the answers

    What is a common nail abnormality seen in individuals with iron-deficiency anemia?

    <p>Koilonychia (spooning of the nails)</p> Signup and view all the answers

    What is the main cause of anemia of chronic disease (ACD)?

    <p>Impaired release of storage iron due to increased hepcidin levels</p> Signup and view all the answers

    Which condition is often associated with Anemia of Chronic Disease (ACD)?

    <p>Systemic lupus erythematosus (SLE)</p> Signup and view all the answers

    What is the primary role of hepcidin in the regulation of body iron?

    <p>Inhibiting the release of storage iron from macrophages</p> Signup and view all the answers

    What type of anemia is commonly associated with normocytic/normochromic or slightly microcytic/hypochromic characteristics?

    <p>Anemia of chronic disease (ACD)</p> Signup and view all the answers

    What is the typical level of serum iron and total iron-binding capacity (TIBC) in Anemia of Chronic Disease (ACD)?

    <p>Low serum iron and normal to increased TIBC</p> Signup and view all the answers

    What is the characteristic feature of sideroblastic anemia?

    <p>Excess iron accumulation in the mitochondrial region of erythrocytes</p> Signup and view all the answers

    Which stain is best used to demonstrate siderocytes in sideroblastic anemia?

    <p>Perl's Prussian blue stain</p> Signup and view all the answers

    What laboratory result is typically observed in sideroblastic anemia?

    <p>Increased ferritin and serum iron</p> Signup and view all the answers

    What distinguishes primary irreversible sideroblastic anemia from the secondary reversible form?

    <p>Known cause of the blocks in heme synthesis</p> Signup and view all the answers

    Which myelodysplastic syndrome is associated with refractory anemia and ringed sideroblasts?

    <p>Refractory anemia with ringed sideroblasts (RARS)</p> Signup and view all the answers

    What factors are known to cause secondary reversible sideroblastic anemia?

    <p>Alcohol, anti-tuberculosis drugs, and chloramphenicol</p> Signup and view all the answers

    Which of the following is a clinical symptom commonly associated with lead poisoning?

    <p>Gum leadline</p> Signup and view all the answers

    In lead poisoning, which of the following is a characteristic laboratory finding related to the red blood cells?

    <p>Normocytic/normochromic anemia with characteristic coarse basophilic stippling</p> Signup and view all the answers

    Which of the following is a symptom NOT commonly associated with lead poisoning in children?

    <p>Confusion</p> Signup and view all the answers

    Which symptom is NOT commonly associated with PORPHYRIAS?

    <p>Hematologic findings</p> Signup and view all the answers

    What is the main cause of the accumulation of heme precursors in the tissues in PORPHYRIAS?

    <p>Block in the protoporphyrin pathway</p> Signup and view all the answers

    What is the primary method of excretion for the large amounts of accumulated heme precursors in PORPHYRIAS?

    <p>Excretion in urine and/or feces</p> Signup and view all the answers

    What is the main cause of megaloblastic anemias?

    <p>Defective DNA synthesis causing abnormal nuclear maturation</p> Signup and view all the answers

    What is the primary cause of the macrocytic/normochromic anemia seen in megaloblastic anemias?

    <p>Defective DNA synthesis affecting nuclear maturation and subsequent erythrocyte development</p> Signup and view all the answers

    Which laboratory finding is characteristic of megaloblastic anemias?

    <p>Elevated LD and bilirubin levels due to destruction of fragile, megaloblastic cells</p> Signup and view all the answers

    What is the primary reason for the presence of hypersegmented neutrophils in megaloblastic anemias?

    <p>Delayed nuclear maturation compared to cytoplasmic maturation</p> Signup and view all the answers

    What is the primary cause of pernicious anemia?

    <p>Deficiency of intrinsic factor</p> Signup and view all the answers

    Which population is most prevalent in pernicious anemia?

    <p>Older adults of English descent</p> Signup and view all the answers

    What is the characteristic symptom of vitamin B12 deficiency?

    <p>Sore tongue (glossitis)</p> Signup and view all the answers

    Which condition is associated with diphyllobothrium latum tapeworm infection as a cause of vitamin B12 deficiency?

    <p>Megaloblastic anemia</p> Signup and view all the answers

    What is the main source of intrinsic factor in the body?

    <p>Stomach parietal cells</p> Signup and view all the answers

    What is the primary cause of pernicious anemia?

    <p>Deficiency of intrinsic factor</p> Signup and view all the answers

    Which ethnic group is particularly prevalent in pernicious anemia?

    <p>English, Irish, and Scandinavian descent</p> Signup and view all the answers

    Which of the following is NOT a cause of vitamin B12 deficiency?

    <p>Consumption of dairy products</p> Signup and view all the answers

    What are the clinical symptoms commonly associated with vitamin B12 deficiency?

    <p>Jaundice and weakness</p> Signup and view all the answers

    What is the primary role of intrinsic factor in vitamin B12 absorption?

    <p>Binding to vitamin B12 for absorption into the intestine</p> Signup and view all the answers

    What is the distinguishing feature of folic acid deficiency anemia?

    <p>No CNS involvement despite clinical symptoms similar to vitamin B12 deficiency</p> Signup and view all the answers

    Which factor is commonly associated with causing folic acid deficiency?

    <p>Pregnancy or poor diet</p> Signup and view all the answers

    What is the consequence of folic acid deficiency on red blood cells?

    <p>Megaloblastic anemia with enlarged, immature red blood cells</p> Signup and view all the answers

    Which condition is most likely to cause non-megaloblastic macrocytic anemia?

    <p>Alcoholism</p> Signup and view all the answers

    What is the characteristic shape of erythrocytes in non-megaloblastic macrocytic anemias?

    <p>Round</p> Signup and view all the answers

    Which of the following conditions is most likely to cause accelerated erythropoiesis and contribute to non-megaloblastic macrocytic anemia?

    <p>Chronic hemolytic anemia</p> Signup and view all the answers

    What is the typical prognosis for patients with aplastic anemia?

    <p>Poor prognosis with complications such as bleeding and iron overload</p> Signup and view all the answers

    What is the primary treatment approach for aplastic anemia?

    <p>Bone marrow or stem cell transplant, and immunosuppression</p> Signup and view all the answers

    At what age group does aplastic anemia most commonly affect people?

    <p>Individuals around the age of 50 and above</p> Signup and view all the answers

    What are the characteristics of genetic aplastic anemia (Fanconi anemia)?

    <p>Autosomal recessive trait, dwarfism, renal disease, mental retardation, strong association with malignancy development</p> Signup and view all the answers

    What is the main characteristic of acquired aplastic anemia?

    <p>Pancytopenia involving all three cell lines</p> Signup and view all the answers

    What distinguishes idiopathic aplastic anemia from genetic and acquired forms?

    <p>It has no known cause and is not associated with any specific genetic or environmental factors</p> Signup and view all the answers

    Which of the following is NOT a known cause of acquired aplastic anemia?

    <p>Vitamin B12 deficiency</p> Signup and view all the answers

    Which of the following is responsible for approximately 30% of acquired aplastic anemias?

    <p>Drug exposure</p> Signup and view all the answers

    Which of the following is NOT commonly associated with secondary acquired aplastic anemia?

    <p>Sideroblastic anemia</p> Signup and view all the answers

    What is the likely cause of Diamond-Blackfan anemia?

    <p>Autosomal dominant inheritance</p> Signup and view all the answers

    What is the primary cause of idiopathic (primary) aplastic anemia?

    <p>Unknown cause</p> Signup and view all the answers

    What is the characteristic feature of true red cell aplasia (Diamond-Blackfan anemia)?

    <p>Normal leukocytes and platelets, but decreased red blood cells</p> Signup and view all the answers

    What is the expected change in reticulocyte count in the initial phase of acute blood loss anemia?

    <p>No change in reticulocyte count</p> Signup and view all the answers

    Which laboratory finding is likely to be observed in the initial few hours after acute blood loss?

    <p>Decrease in platelet count and leukocytosis with a left shift</p> Signup and view all the answers

    What is the characteristic feature of normocytic/normochromic anemia in acute blood loss anemia?

    <p>Normal cell size and normal hemoglobin content</p> Signup and view all the answers

    What type of anemia is characterized by a gradual, long-term loss of blood?

    <p>Chronic blood loss anemia</p> Signup and view all the answers

    What type of anemia is initially normocytic/normochromic but over time causes a decrease in hemoglobin/hematocrit?

    <p>Normocytic/normochromic anemia</p> Signup and view all the answers

    Which type of anemia results from a gradual loss of iron causing microcytic/hypochromic anemia?

    <p>Chronic blood loss anemia</p> Signup and view all the answers

    What is the underlying cause of hemolytic anemias due to intrinsic defects?

    <p>Hereditary with reticulocytosis due to accelerated destruction</p> Signup and view all the answers

    What is the characteristic type of anemia seen in hemolytic anemias due to intrinsic defects?

    <p>Normocytic/normochromic anemia</p> Signup and view all the answers

    What is the primary distinguishing feature of hemolytic anemias due to intrinsic defects?

    <p>Accelerated destruction of erythrocytes with reticulocytosis</p> Signup and view all the answers

    Which gene is most commonly affected in hereditary spherocytosis?

    <p>ANK1</p> Signup and view all the answers

    What is the primary reason for the need of splenectomy in patients with hereditary spherocytosis?

    <p>Removal of rigid spherocytes by splenic macrophages</p> Signup and view all the answers

    What is the characteristic finding on a peripheral blood film in hereditary spherocytosis?

    <p>Spherocytes</p> Signup and view all the answers

    Which laboratory finding supports the diagnosis of hereditary spherocytosis?

    <p>Decreased osmotic fragility</p> Signup and view all the answers

    What does the Osmotic fragility test indicate?

    <p>The presence of spherocytes</p> Signup and view all the answers

    What is the result of the Direct Antiglobulin Test (DAT) in Hereditary Spherocytosis (HS)?

    <p>It is negative in HS</p> Signup and view all the answers

    What is the primary limitation of the Osmotic fragility test in distinguishing Hereditary Spherocytosis (HS) from other causes of spherocytes?

    <p>It is less sensitive and specific</p> Signup and view all the answers

    What is the defining characteristic of hereditary elliptocytosis/hereditary ovalocytosis?

    <p>Mutation in the spectrin α chain</p> Signup and view all the answers

    In which condition are elliptocytes defined as cells that are twice as long as they are wide?

    <p>Hereditary elliptocytosis/hereditary ovalocytosis</p> Signup and view all the answers

    What is the diagnostic threshold for the percentage of circulating red cells that are elliptocytes in hereditary elliptocytosis/hereditary ovalocytosis?

    <p>More than 25%</p> Signup and view all the answers

    What is the defining characteristic used to diagnose hereditary elliptocytosis/hereditary ovalocytosis?

    <p>More than 25% of circulating red cells are elliptocytes</p> Signup and view all the answers

    Which condition commonly shows low proportions of elliptocytes and can be mistaken for hereditary elliptocytosis/hereditary ovalocytosis?

    <p>Iron deficiency anemia</p> Signup and view all the answers

    What is the primary cause of the characteristic cell shape seen in hereditary elliptocytosis/hereditary ovalocytosis?

    <p>Mutation in the spectrin α chain</p> Signup and view all the answers

    What is the primary distinguishing characteristic of hereditary pyropoikilocytosis (HPP)?

    <p>Unusual sensitivity of red cells to heat</p> Signup and view all the answers

    What type of anemia is associated with moderate to severe anemia in homozygotes?

    <p>Hereditary pyropoikilocytosis (HPP)</p> Signup and view all the answers

    Which double heterozygosity leads to the spherocytic type of anemia?

    <p>HS and HE</p> Signup and view all the answers

    What is the primary cause of the variable degree of anemia in hereditary stomatocytosis?

    <p>Altered membrane defect leading to abnormal permeability to both sodium and potassium</p> Signup and view all the answers

    What is the main effect of the membrane defect in hereditary stomatocytosis on erythrocytes?

    <p>Results in abnormal swelling of erythrocytes</p> Signup and view all the answers

    What is the quantitative range of stomatocytes typically observed on the blood smear in hereditary stomatocytosis?

    <p>50-75%</p> Signup and view all the answers

    What are the defining characteristics of hereditary stomatocytosis severe hydrocytotic (overhydrated) type?

    <p>Macrocytosis, moderate to severe hemolysis, low MCHC</p> Signup and view all the answers

    What are the characteristic features of xerocytotic type hereditary stomatocytosis?

    <p>Normocytic RBCs seen as spiculated dessicocytes, moderate stomatocytosis, and target cells</p> Signup and view all the answers

    What is the most likely blood smear observation in hereditary stomatocytosis severe hydrocytotic (overhydrated) type?

    <p>Stomatocytes and target cells</p> Signup and view all the answers

    What is the primary cause of the neurological and retinal abnormalities associated with hereditary acanthocytosis?

    <p>Absence of serum p-lipoprotein for lipid transport</p> Signup and view all the answers

    What is the main factor responsible for the increased cholesterol:lecithin ratio in the erythrocyte membrane in hereditary acanthocytosis?

    <p>Decreased serum p-lipoprotein for lipid transport</p> Signup and view all the answers

    What is the characteristic feature of the erythrocyte membrane in hereditary acanthocytosis?

    <p>Increased cholesterol:lecithin ratio</p> Signup and view all the answers

    What is the primary cause of hemolytic anemia in G6PD deficiency?

    <p>Inadequate NADPH generation</p> Signup and view all the answers

    What is the characteristic denaturation form seen in G6PD deficiency-induced hemolytic anemia?

    <p>Heinz bodies</p> Signup and view all the answers

    In G6PD deficiency, when does severe hemolytic anemia with reticulocytosis usually occur?

    <p>When oxidatively challenged</p> Signup and view all the answers

    What is the primary mechanism behind the severe hemolytic anemia seen in pyruvate kinase (PK) deficiency?

    <p>Impairment of the cation pump controlling intracellular sodium and potassium levels</p> Signup and view all the answers

    How does the lack of ATP in pyruvate kinase (PK) deficiency affect the erythrocyte life span?

    <p>Shortens the erythrocyte life span</p> Signup and view all the answers

    How does the impairment of the cation pump affect the intracellular environment of erythrocytes in pyruvate kinase (PK) deficiency?

    <p>Leads to decreased levels of intracellular sodium and potassium</p> Signup and view all the answers

    What is the most common membrane defect in hereditary spherocytosis?

    <p>Defect in ankyrin protein</p> Signup and view all the answers

    What is the effect of increased permeability of the membrane to sodium in hereditary spherocytosis?

    <p>Increased osmotic fragility of erythrocytes</p> Signup and view all the answers

    What is the primary reason for affected patients requiring splenectomy in hereditary spherocytosis?

    <p>Culling/Removal of rigid spherocytes by splenic macrophages</p> Signup and view all the answers

    What is the most common affected gene in hereditary spherocytosis, after ANK1 (ankyrin)?

    <p>Band 3 protein (SLC4A1)</p> Signup and view all the answers

    What is the defining characteristic of hereditary elliptocytosis/hereditary ovalocytosis?

    <p>At least 25% of circulating red cells are elliptocytes</p> Signup and view all the answers

    What is a possible cause for low proportions of elliptocytes seen in the blood?

    <p>Iron deficiency</p> Signup and view all the answers

    What laboratory test is less sensitive and specific for distinguishing hereditary spherocytosis from other causes of spherocytes?

    <p>Osmotic fragility test</p> Signup and view all the answers

    What result would be expected from a direct antiglobulin test (DAT) in hereditary spherocytosis?

    <p>-ve (negative)</p> Signup and view all the answers

    What is the primary effect of the membrane defect in hereditary stomatocytosis on erythrocytes?

    <p>Decreased osmotic fragility of erythrocytes</p> Signup and view all the answers

    What does a diagnosis of Hereditary Elliptocytosis/Hereditary Ovalocytosis entail?

    <p>Presence of at least 25% elliptocytes in circulating red cells</p> Signup and view all the answers

    What is indicative if gel electrophoresis of red cell membrane proteins is used to support diagnosis?

    <p>Diagnosing hereditary spherocytosis</p> Signup and view all the answers

    What is the most common enzyme deficiency in the hexose monophosphate shunt?

    <p>G6PD (GLUCOSE-6-PHOSPHATE DEHYDROGENASE) DEFICIENCY</p> Signup and view all the answers

    Which condition results in oxidation of hemoglobin to methemoglobin (Fe3+)?

    <p>G6PD (GLUCOSE-6-PHOSPHATE DEHYDROGENASE) DEFICIENCY</p> Signup and view all the answers

    Which deficiency causes impairment of the cation pump that controls intracellular sodium and potassium levels?

    <p>PYRUVATE KINASE (PK) DEFICIENCY</p> Signup and view all the answers

    Which condition is characterized by chronic intravascular hemolysis causing hemoglobinuria and hemosiderinuria at an acid pH at night?

    <p>PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH)</p> Signup and view all the answers

    Which deficiency results in decreased NADPH generation, leading to reduced glutathione levels?

    <p>G6PD (GLUCOSE-6-PHOSPHATE DEHYDROGENASE) DEFICIENCY</p> Signup and view all the answers

    What is the primary cause of severe hemolytic anemia with reticulocytosis in PYRUVATE KINASE (PK) DEFICIENCY?

    <p>Decreased erythrocyte deformability</p> Signup and view all the answers

    In which condition are all cells abnormally sensitive to lysis by complement?

    <p>PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH)</p> Signup and view all the answers

    What characteristic is used in the diagnosis of PAROXYSMAL NOCTURNAL HEMOGLOBINURIA (PNH)?

    <p>Low leukocyte alkaline phosphatase (LAP) score</p> Signup and view all the answers

    What effect does PYRUVATE KINASE (PK) DEFICIENCY have on erythrocyte deformability?

    <p>Decreases deformability</p> Signup and view all the answers

    Which mechanism is responsible for intravascular hemolysis in ABO incompatibility?

    <p>Complement-induced immune response</p> Signup and view all the answers

    What is the primary cause of hemolytic disease of the newborn (HDN) due to Rh incompatibility?

    <p>Development of IgG antibodies</p> Signup and view all the answers

    What is the characteristic laboratory finding in hemolytic disease of the newborn (HDN) due to ABO incompatibility?

    <p>Weakly positive direct antiglobulin test (DAT)</p> Signup and view all the answers

    What is the primary cause of severe anemia and high bilirubin levels in hemolytic disease of the newborn (HDN) due to Rh incompatibility?

    <p>Alloimmune destruction of fetal RBCs</p> Signup and view all the answers

    Which condition has become less common due to the use of Rh immunoglobulin (RhoGam)?

    <p>Hemolytic disease of the newborn (HDN) due to Rh incompatibility</p> Signup and view all the answers

    What is the underlying cause of hemolytic transfusion reaction?

    <p>Donor cells coated with recipient's antibodies</p> Signup and view all the answers

    Which test is typically positive in hemolytic transfusion reaction?

    <p>Direct Coombs test (DAT)</p> Signup and view all the answers

    What is the likely laboratory finding in hemolytic transfusion reaction?

    <p>Decreased plasma hemoglobin</p> Signup and view all the answers

    Which condition can trigger disseminated intravascular coagulation (DIC) due to release of tissue factor from lysed RBCs?

    <p>Hemolytic disease of the newborn (HDN) due to ABO incompatibility</p> Signup and view all the answers

    In warm autoimmune hemolytic anemia (WAIHA), what is the primary antibody coating the RBCs?

    <p>IgG</p> Signup and view all the answers

    Which condition can lead to cold autoimmune hemolytic anemia (CAIHA)?

    <p>Infectious mononucleosis</p> Signup and view all the answers

    What is the defining characteristic of paroxysmal cold hemoglobinuria (PCH)?

    <p>Presence of IgM biphasic Donath-Landsteiner antibody</p> Signup and view all the answers

    What is a common secondary cause of warm autoimmune hemolytic anemia (WAIHA)?

    <p>Rheumatoid arthritis</p> Signup and view all the answers

    What laboratory finding is commonly associated with cold autoimmune hemolytic anemia (CAIHA)?

    <p>RBC clumping at high temperatures</p> Signup and view all the answers

    How does the complement-coated RBC in cold autoimmune hemolytic anemia (CAIHA) typically react when warmed to 37°C?

    <p>Undergoes intravascular hemolysis</p> Signup and view all the answers

    What is the primary temperature range at which red blood cell clumping is observed in cold autoimmune hemolytic anemia (CAIHA)?

    <p>-5°C to 5°C</p> Signup and view all the answers

    What is the primary effect of the IgG biphasic Donath-Landsteiner antibody in paroxysmal cold hemoglobinuria (PCH)?

    <p>Fixes complement to RBCs in warm conditions</p> Signup and view all the answers

    In warm autoimmune hemolytic anemia (WAIHA), how are red blood cells (RBCs) coated?

    <p>With IgG and/or complement</p> Signup and view all the answers

    What is the characteristic antibody involved in cold autoimmune hemolytic anemia (CAIHA)?

    <p>Anti-I</p> Signup and view all the answers

    Which condition is associated with the presence of IgG biphasic Donath-Landsteiner antibody with P specificity?

    <p>Paroxysmal cold hemoglobinuria (PCH)</p> Signup and view all the answers

    What is the characteristic finding in the laboratory related to RBC clumping in cold autoimmune hemolytic anemia (CAIHA)?

    <p>Positive direct antiglobulin test (DAT)</p> Signup and view all the answers

    Which disease can secondary cold autoimmune hemolytic anemia (CAIHA) be associated with?

    <p>Infectious mononucleosis</p> Signup and view all the answers

    In warm autoimmune hemolytic anemia (WAIHA), what can macrophages do to red blood cells (RBCs) coated with IgG and/or complement?

    <p>They may phagocytize these RBCs, or remove the antibody or complement from the RBC's surface</p> Signup and view all the answers

    What causes the lysis of red blood cells (RBCs) in paroxysmal cold hemoglobinuria (PCH)?

    <p>Complement fixation at temperatures below 37°C</p> Signup and view all the answers

    What is the primary cause of cold autoimmune hemolytic anemia (CAIHA)?

    <p>Defects extrinsic to the RBC</p> Signup and view all the answers

    In warm autoimmune hemolytic anemia (WAIHA), what can be seen in 60% of cases?

    <p>'Idiopathic cases'</p> Signup and view all the answers

    Which condition is characterized by systemic clotting initiated by activation of the coagulation cascade due to toxins or conditions that trigger release of procoagulants?

    <p>Disseminated intravascular coagulation</p> Signup and view all the answers

    Which condition most often occurs in children following a gastrointestinal infection and causes clots to form, leading to renal damage?

    <p>Hemolytic uremic syndrome</p> Signup and view all the answers

    What type of anemia does March hemoglobinuria cause following forceful contact of the body with hard surfaces, such as in marathon runners or tennis players?

    <p>Infectious agent-induced anemia</p> Signup and view all the answers

    In which type of anemia are schistocytes and spherocytes seen on the blood smear?

    <p>Microangiopathic hemolytic anemias</p> Signup and view all the answers

    What causes acute hemolysis and severe anemia with many schistocytes and micro-spherocytes?

    <p>Thermal burns (third degree)</p> Signup and view all the answers

    What causes fibrin to be deposited in small vessels, leading to RBC fragmentation?

    <p>Activation of the coagulation cascade</p> Signup and view all the answers

    What is the primary cause of MARCH HEMOGLOBINURIA?

    <p>Physical trauma</p> Signup and view all the answers

    Which factor triggers the initiation of systemic clotting in Disseminated Intravascular Coagulation (DIC)?

    <p>Toxins triggering release of procoagulants</p> Signup and view all the answers

    Which condition is characterized by intracellular rodlike C crystals?

    <p>Hemoglobin C disease (Hgb CC)</p> Signup and view all the answers

    What is the cause of Hgb E?

    <p>Lysine replacement of glutamic acid at position 26 on the beta chain</p> Signup and view all the answers

    In which populations is Hemoglobin E found more commonly?

    <p>Southeast Asian, African, and African-American populations</p> Signup and view all the answers

    What is the life expectancy with proper treatment for individuals diagnosed with sickle cell anemia?

    <p>50 years</p> Signup and view all the answers

    What is the most common hemoglobinopathy in the United States?

    <p>Sickle cell trait (Hgb AS)</p> Signup and view all the answers

    What is the characteristic laboratory finding in individuals with Hgb SC disease?

    <p>Rare sickle cells or C crystals</p> Signup and view all the answers

    What migrates with hemoglobins A2, E, and O on alkaline hemoglobin electrophoresis in individuals with Hemoglobin C disease?

    <p>Hemoglobin A2</p> Signup and view all the answers

    What is the approximate proportion of Hgb S and Hgb C produced in individuals with Hgb SC disease?

    <p>~50% Hgb S and ~50% Hgb C</p> Signup and view all the answers

    In individuals with sickle cell trait (Hgb AS), what is the approximate percentage of Hgb A and Hgb S produced?

    <p>~60% Hgb A and ~40% Hgb S</p> Signup and view all the answers

    What migrates with hemoglobin D on alkaline hemoglobin electrophoresis?

    <p>Hemoglobin G</p> Signup and view all the answers

    What is the characteristic laboratory finding in individuals with sickle cell anemia (Hgb SS)?

    <p>Target cells</p> Signup and view all the answers

    What is the primary cause of death in individuals diagnosed with sickle cell anemia?

    <p>Infection</p> Signup and view all the answers

    What is the primary cause of sickle cell disease?

    <p>Mutation in the beta globin chain</p> Signup and view all the answers

    Which population is sickle cell disease most commonly found in?

    <p>African-American, African, Mediterranean, and Middle Eastern</p> Signup and view all the answers

    What is the composition of hemoglobin in sickle cell disease?

    <p>100% Hgb S</p> Signup and view all the answers

    What is the characteristic shape of erythrocytes in sickle cell disease?

    <p>Sickle-shaped</p> Signup and view all the answers

    What is the consequence of erythrocytes becoming rigid and trapped in capillaries in sickle cell disease?

    <p>Tissue necrosis due to lack of oxygen delivery</p> Signup and view all the answers

    Which laboratory test may be used to confirm the diagnosis of sickle cell disease?

    <p>Hemoglobin electrophoresis, isoelectric focusing and/or DNA (PCR) analysis</p> Signup and view all the answers

    What is the effect of sickle cell disease on hemoglobin solubility and function?

    <p>Decrease in solubility and function</p> Signup and view all the answers

    What is the primary difference between homozygous/disease conditions and heterozygous/trait conditions in hemoglobinopathies?

    <p>'Homozygous/disease' affects both globin chains, while 'heterozygous/trait' affects only one globin chain</p> Signup and view all the answers

    What are target cells associated with in the context of hemoglobinopathies?

    <p>'Homozygous/disease' conditions</p> Signup and view all the answers

    What is the most common amino acid substitution causing formation of Hgb S?

    <p>'Valine replaces glutamic acid at position 6'</p> Signup and view all the answers

    What occurs when deoxyhemoglobin forms in sickle cell disease?

    <p>Hemoglobin crystallizes within erythrocytes.</p> Signup and view all the answers

    What is the consequence of sickled erythrocytes being trapped in capillaries?

    <p>Tissue necrosis due to lack of oxygen delivery.</p> Signup and view all the answers

    What is the primary characteristic of thalassemia minor/trait?

    <p>Sufficient alpha and beta chains produced to make normal hemoglobin A, A2, and F</p> Signup and view all the answers

    What is the hallmark laboratory finding in beta-thalassemia major (Cooley anemia)?

    <p>Increased serum iron and increased bilirubin</p> Signup and view all the answers

    What characterizes the red blood cell (RBC) membrane in beta-thalassemia major?

    <p>Formation of Heinz bodies causing rigidity</p> Signup and view all the answers

    What is the primary reason for stunted growth in individuals with beta-thalassemia major?

    <p>Iron overload from RBC destruction</p> Signup and view all the answers

    What is the characteristic appearance of RBCs in the laboratory findings of beta-thalassemia major?

    <p>Microcytic/hypochromic RBCs with target cells</p> Signup and view all the answers

    What is the primary effect of the excess alpha chains in beta-thalassemia major?

    <p>Precipitation on the RBC membrane causing rigidity</p> Signup and view all the answers

    What is the primary cause of organ failure in individuals with beta-thalassemia major?

    <p>(Hgb F) production greater than 90%</p> Signup and view all the answers

    What symptoms are likely to be present by 6 months of age in individuals with beta-thalassemia major?

    <p>(Hepatosplenomegaly) and (jaundice)</p> Signup and view all the answers

    Which condition does NOT cause a decreased rate of synthesis of a structurally normal globin chain?

    <p>Hereditary spherocytosis</p> Signup and view all the answers

    In which population is thalassemia primarily found?

    <p>Asian</p> Signup and view all the answers

    In alpha-thalassemia major (hydrops fetalis), what is the primary reason for the incompatibility with life?

    <p>Deletion of all four alpha genes leading to no normal hemoglobin production</p> Signup and view all the answers

    What distinguishes alpha-thalassemia HgbH disease from other types of alpha-thalassemia?

    <p>Moderate microcytic/hypochromic anemia and up to 30% Hgb H produced</p> Signup and view all the answers

    What is a distinguishing characteristic of alpha-thalassemia minor/trait?

    <p>Mild microcytic/hypochromic anemia often with a high RBC count and target cells</p> Signup and view all the answers

    What distinguishes alpha-thalassemia silent carrier from other types of alpha-thalassemia?

    <p>Deletion of one alpha gene and asymptomatic patients</p> Signup and view all the answers

    What happens in alpha-thalassemia HgbH disease that leads to the formation of Heinz bodies and the destruction of rigid RBCs in the spleen?

    <p>Decrease in alpha chains leading to beta chain excess</p> Signup and view all the answers

    What is the primary reason for asymptomatic patients in alpha-thalassemia silent carrier?

    <p>Deletion of one alpha gene and asymptomatic patients</p> Signup and view all the answers

    Study Notes

    Erythrocyte Lifespan and Dimensions

    • Normal lifespan of erythrocytes is about 120 days.
    • Erythrocytes’ approximate diameter ranges from 6 to 8 micrometers.
    • Central pallor size of erythrocytes is about one-third of the cell’s diameter.
    • Average diameter of erythrocytes is larger than the nucleus of a small lymphocyte.

    Erythropoiesis and Regulation

    • Erythropoietin stimulates myeloid progenitor cells to produce erythrocytes and influences CFU-Es to differentiate into erythroblasts.
    • Erythropoietin is mainly produced in the kidneys.
    • Growth factors besides erythropoietin regulating erythropoiesis include androgens.
    • Reference range of red blood cell count for males is approximately 4.7 to 6.1 million cells per microliter.

    Normoblasts and Reticulocytes

    • Hemoglobin production in reticulocytes continues for approximately 24 to 48 hours after exiting the bone marrow.
    • Polychromatophilic normoblast (rubricyte) size ranges from 10 to 15 micrometers.
    • Pronormoblast (rubriblast) has a N:C ratio of about 8:1.
    • Eccentric nucleus with small, fully condensed (pyknotic) nucleus describes the orthochromic normoblast (metarubricyte).
    • Normoblast producing hemoglobin has a gray-blue cytoplasm.

    Red Blood Cell Characteristics

    • Microcytes are red blood cells less than 6 micrometers in diameter; macrocytes are greater than 8 micrometers in diameter.
    • Normocytes (discocytes) have a biconcave shape and a central pallor.
    • Anisocytosis indicates variation in size among red blood cells.
    • Increased RDW (red blood cell distribution width) is observed in various anemias, indicating size variability among RBCs.

    Poikilocytosis and Cell Shapes

    • Poikilocytosis reflects variations in cell shape among red blood cells.
    • Conditions with acanthocytes show irregular projections on the cell's surface.
    • Echinocytes (burr cells) result from membrane changes and increased surface-to-volume ratio.
    • Target cells (codocytes) are associated with certain types of anemia, such as thalassemia.

    Abnormal Cell Forms and Anemias

    • Sickle cells are commonly seen in sickle cell anemia and result from altered hemoglobin structure.
    • Schistocytes (RBC fragments) arise in microangiopathic hemolytic anemias due to mechanical fragmentation.
    • Pear-shaped cells are characteristic of porphyrias.
    • Stomatocytes (mouth cells) have a slit-like central area and are associated with liver disease.

    Nucleated Red Blood Cells

    • Nucleated RBCs are seen in bone marrow stress and can indicate various pathological conditions.
    • Presence of Howell-Jolly bodies often suggests splenic dysfunction or postsplenectomy.
    • Cabot rings indicate disturbed erythropoiesis and may appear in certain anemias.

    Hemoglobin and Crystals

    • Hemoglobin is essential for oxygen transport and consists of four heme groups per molecule.
    • Hemoglobin SC crystals (Washington monument) are found in sickle cell disease with hemoglobin C.
    • Heinz bodies indicate oxidative damage to RBCs and can be seen in G6PD deficiency and other conditions.

    Staining and Microscopic Features

    • Basophilic stippling reflects lead poisoning or thalassemia, resulting from ribosomal RNA aggregation.
    • The characteristic appearance of Pappenheimer bodies reflects iron accumulation in red blood cells.
    • Variation in staining intensity indicates hypochromasia or normochromasia, often associated with microcytic anemias.

    Agglutination and Rouleaux Formation

    • Agglutination in cold autoimmune hemolytic anemia leads to form clusters of RBCs known as agglutinates.
    • Rouleaux formation suggests increased plasma protein concentration, often seen in inflammatory conditions.
    • Careful interpretation of RBC counts is necessary in cold agglutinin disease to avoid misclassification.

    General Knowledge

    • Oxygen transport is vital to cell metabolism and is facilitated by hemoglobin’s iron-containing heme groups.
    • Erythrocytes derive their red color primarily from hemoglobin.
    • The amino acid sequence in hemoglobin's globin chain determines its type and functionality.

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    Test your knowledge about the structure and function of erythrocytes, including their size, shape, life span, and the role of erythropoietin in stimulating their production. Explore the essential role of erythrocytes in oxygen transport and removal of metabolic waste.

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