Pelger-Huët Anomaly Quiz
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Questions and Answers

What is the characteristic nucleus shape of Pelger-Huët anomaly?

  • Spherical
  • Peanut-shaped (correct)
  • Ring-shaped
  • Multilobed

    • Which of the following is a characteristic of Pseudo-Pelger-Huët anomaly?

  • Relatively lower N:C ratio
  • More affected cells
  • Darker, more coarse chromatin
  • Larger cell size (correct)

    • What is the name of the darkly stained structure in the nucleus of female cells?

  • Centriole
  • Nucleolus
  • Barr body (correct)
  • Lampbrush chromosome

    • Which of the following is a characteristic of Auer rods?

    <p>Pink or red stained needle-like crystals</p> Signup and view all the answers

    What is the name of the cells that are usually larger and rounder in shape?

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

    What is the % of myeloid bias in Pelger-Huët anomaly?

    <p>63-93%</p> Signup and view all the answers

    What is the main cause of thrombocytopenia in May-Hegglin anomaly?

    <p>Mutation in the MYH9 gene affecting megakaryocyte maturation</p> Signup and view all the answers

    What is the characteristic feature of Döhle bodies in May-Hegglin anomaly?

    <p>They are larger and rounder in shape</p> Signup and view all the answers

    Which of the following is NOT a type of Lysosomal Storage Disease?

    <p>Mitochondrial myopathies</p> Signup and view all the answers

    What is the approximate platelet count in May-Hegglin anomaly?

    <p>40-80 x 10^9/L</p> Signup and view all the answers

    What is the composition of the inclusion in the red arrow?

    <p>Myosin Heavy Chain Type IIA</p> Signup and view all the answers

    What is the underlying defect in Lysosomal Storage Diseases?

    <p>Flawed degradation of phagocytized material</p> Signup and view all the answers

    What percentage of cases of X-linked Chronic Granulomatous Disease (CGD) account for the most severe symptoms?

    <p>70%</p> Signup and view all the answers

    What is the main problem in X-linked Chronic Granulomatous Disease (CGD)?

    <p>Decreased ability of phagocytes to produce superoxide and reactive oxygen species</p> Signup and view all the answers

    What is the genetic defect that results in the CGD phenotype?

    <p>Mutation in genes responsible for proteins that make up a complex known as NADPH oxidase</p> Signup and view all the answers

    What is the purpose of the NADPH oxidase enzyme in a normal setting?

    <p>To form hydrogen peroxide with the addition of water</p> Signup and view all the answers

    What is the name of the test used to check if a WBC is normal or has CGD?

    <p>NITROBLUE TETRAZOLIUM REDUCTION TEST</p> Signup and view all the answers

    What is the characteristic finding in Lazy Leukocyte Syndrome?

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

    What is the primary defect in Schwachman Syndrome?

    <p>Defective actin filaments in neutrophils</p> Signup and view all the answers

    What is the primary reason for increased hematocrit at high altitudes?

    <p>To compensate for decreased oxygen in the air</p> Signup and view all the answers

    What is the consequence of defective NADPH oxidase in neutrophils?

    <p>Decreased ability of phagocytes to produce superoxide and reactive oxygen species</p> Signup and view all the answers

    What is the characteristic of catalase-positive microorganisms?

    <p>They are able to break down H2O2</p> Signup and view all the answers

    What is the primary functionally definition of anemia?

    <p>A decrease in the oxygen carrying capacity of the blood</p> Signup and view all the answers

    What is the result of a normal Nitroblue Tetrazolium test?

    <p>The yellow substance is reduced to a dark blue insoluble formazan</p> Signup and view all the answers

    What is the significance of petechiae in a patient's skin?

    <p>It indicates a hematologic disorder</p> Signup and view all the answers

    What is the characteristic of moderate anemia?

    <p>Hemoglobin concentration of 7-10 g/dL</p> Signup and view all the answers

    What is the primary problem in disorders related to a defect in microbicidal activity?

    <p>Decreased ability of phagocytes to kill engulfed microorganisms</p> Signup and view all the answers

    What is the mode of inheritance of defective locomotion/mobility in neutrophils?

    <p>X-linked, but some autosomal recessive or point mutations</p> Signup and view all the answers

    What is the characteristic of megaloblastic anemia?

    <p>Oval macrocyte, large oval RBC</p> Signup and view all the answers

    What is the significance of jaundice in a patient with anemia?

    <p>It indicates increased RBC destruction</p> Signup and view all the answers

    What is the main reason for the apparent decrease in red blood cell count in relative anemia?

    <p>Dilution of red blood cells due to increased plasma volume</p> Signup and view all the answers

    What is the characteristic of erythrocytosis?

    <p>Increased red blood cell count</p> Signup and view all the answers

    What is the result of a shift of fluid from extracellular to intracellular compartments?

    <p>Dilution of red blood cells</p> Signup and view all the answers

    What is the difference between relative anemia and true anemia?

    <p>Relative anemia is characterized by a dilution effect due to increased plasma volume, while true anemia is characterized by a true decrease in red blood cell mass</p> Signup and view all the answers

    What is the characteristic of relative erythrocytosis?

    <p>An apparent increase in red blood cell concentration due to decreased plasma volume</p> Signup and view all the answers

    What is the characteristic of bone marrow in iron deficiency anemia?

    <p>Abundant iron stores in macrophages but not RBC precursors</p> Signup and view all the answers

    What is the purpose of measuring sTfRs in serum in diagnosing iron deficiency anemia?

    <p>To distinguish iron deficiency from anemia of chronic inflammation</p> Signup and view all the answers

    What is the significance of the Thomas plot in diagnosing iron deficiency anemia?

    <p>It distinguishes iron deficiency from anemia of chronic inflammation</p> Signup and view all the answers

    What is the typical duration of iron replacement therapy?

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

    What happens to the products from the earlier stages in the pathway of heme synthesis when an enzyme is missing?

    <p>They accumulate in the cells</p> Signup and view all the answers

    What is the characteristic of the porphyrias that have hematologic manifestations?

    <p>Only three of them have hematologic manifestations</p> Signup and view all the answers

    What is the benefit of using oral bovine lactoferrin for iron supplementation?

    <p>It has reduced intestinal side effects compared to ferrous sulfate</p> Signup and view all the answers

    In which cases is intravenous administration of iron dextrans used?

    <p>In rare cases with impaired intestinal absorption of iron</p> Signup and view all the answers

    What is the consequence of the deposition of accumulated products in the skin?

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

    What is the timeframe for reticulocyte counts to begin to increase after optimal iron treatment?

    <p>5-10 days</p> Signup and view all the answers

    What is a common side effect of iron supplementation that can lead to poor patient compliance?

    <p>All of the above</p> Signup and view all the answers

    Why is RBC transfusion rarely warranted for the correction of uncomplicated iron deficiency?

    <p>Because of the risks associated with RBC transfusions</p> Signup and view all the answers

    What is the primary cause of chronic inflammation-related anemia?

    <p>Impaired ferrokinetics</p> Signup and view all the answers

    How do inflammatory cytokines affect erythroid progenitor cells?

    <p>They decrease proliferation and responsiveness to erythropoietin</p> Signup and view all the answers

    What is the effect of high hepcidin on macrophages and hepatocytes?

    <p>They cannot export iron to erythroblasts</p> Signup and view all the answers

    What is the role of lactoferrin in iron acquisition by erythroblasts?

    <p>Erythroblasts cannot acquire iron from lactoferrin</p> Signup and view all the answers

    What is the effect of chronic inflammation on RBC life span?

    <p>It decreases RBC life span</p> Signup and view all the answers

    What is the role of hemophagocytic macrophages in chronic inflammation-related anemia?

    <p>They suppress erythropoiesis</p> Signup and view all the answers

    What is the normal role of HFE in regulating iron absorption?

    <p>To reduce iron absorption by triggering hepcidin production</p> Signup and view all the answers

    What is the effect of ferrous iron accumulation in cells?

    <p>It leads to the formation of reactive oxygen species</p> Signup and view all the answers

    What is the consequence of mutated HFE not binding to B2-microglobulin?

    <p>It leads to increased iron absorption</p> Signup and view all the answers

    What is the role of transferrin receptor 1 in the regulation of iron absorption?

    <p>It binds to HFE to trigger hepcidin production</p> Signup and view all the answers

    What is the result of excess iron in parenchymal cells?

    <p>It leads to the formation of ferritin and hemosiderin</p> Signup and view all the answers

    What is the normal function of HFE in the cell?

    <p>To bind to B2-microglobulin to regulate iron absorption</p> Signup and view all the answers

    What is the purpose of lab testing in hemochromatosis?

    <p>All of the above</p> Signup and view all the answers

    What may be elevated in individuals with hereditary hemochromatosis?

    <p>Serum ferritin and alanine transaminase level</p> Signup and view all the answers

    What is essential to determine the degree of organ damage in hemochromatosis?

    <p>Liver biopsy and assessment of iron staining</p> Signup and view all the answers

    Why may individuals with undiagnosed hereditary hemochromatosis come to medical attention?

    <p>Due to organ function problems leading to specific physical complaints</p> Signup and view all the answers

    What can be used to monitor treatment in hemochromatosis?

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

    What can be used as a screening test for hereditary hemochromatosis?

    <p>Elevations of transferrin saturation or serum ferritin</p> Signup and view all the answers

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