65 Questions
What is the characteristic nucleus shape of Pelger-Huët anomaly?
Peanut-shaped
Which of the following is a characteristic of Pseudo-Pelger-Huët anomaly?
Larger cell size
What is the name of the darkly stained structure in the nucleus of female cells?
Barr body
Which of the following is a characteristic of Auer rods?
Pink or red stained needle-like crystals
What is the name of the cells that are usually larger and rounder in shape?
Faggot cells
What is the % of myeloid bias in Pelger-Huët anomaly?
63-93%
What is the main cause of thrombocytopenia in May-Hegglin anomaly?
Mutation in the MYH9 gene affecting megakaryocyte maturation
What is the characteristic feature of Döhle bodies in May-Hegglin anomaly?
They are larger and rounder in shape
Which of the following is NOT a type of Lysosomal Storage Disease?
Mitochondrial myopathies
What is the approximate platelet count in May-Hegglin anomaly?
40-80 x 10^9/L
What is the composition of the inclusion in the red arrow?
Myosin Heavy Chain Type IIA
What is the underlying defect in Lysosomal Storage Diseases?
Flawed degradation of phagocytized material
What percentage of cases of X-linked Chronic Granulomatous Disease (CGD) account for the most severe symptoms?
70%
What is the main problem in X-linked Chronic Granulomatous Disease (CGD)?
Decreased ability of phagocytes to produce superoxide and reactive oxygen species
What is the genetic defect that results in the CGD phenotype?
Mutation in genes responsible for proteins that make up a complex known as NADPH oxidase
What is the purpose of the NADPH oxidase enzyme in a normal setting?
To form hydrogen peroxide with the addition of water
What is the name of the test used to check if a WBC is normal or has CGD?
NITROBLUE TETRAZOLIUM REDUCTION TEST
What is the characteristic finding in Lazy Leukocyte Syndrome?
Neutropenia
What is the primary defect in Schwachman Syndrome?
Defective actin filaments in neutrophils
What is the primary reason for increased hematocrit at high altitudes?
To compensate for decreased oxygen in the air
What is the consequence of defective NADPH oxidase in neutrophils?
Decreased ability of phagocytes to produce superoxide and reactive oxygen species
What is the characteristic of catalase-positive microorganisms?
They are able to break down H2O2
What is the primary functionally definition of anemia?
A decrease in the oxygen carrying capacity of the blood
What is the result of a normal Nitroblue Tetrazolium test?
The yellow substance is reduced to a dark blue insoluble formazan
What is the significance of petechiae in a patient's skin?
It indicates a hematologic disorder
What is the characteristic of moderate anemia?
Hemoglobin concentration of 7-10 g/dL
What is the primary problem in disorders related to a defect in microbicidal activity?
Decreased ability of phagocytes to kill engulfed microorganisms
What is the mode of inheritance of defective locomotion/mobility in neutrophils?
X-linked, but some autosomal recessive or point mutations
What is the characteristic of megaloblastic anemia?
Oval macrocyte, large oval RBC
What is the significance of jaundice in a patient with anemia?
It indicates increased RBC destruction
What is the main reason for the apparent decrease in red blood cell count in relative anemia?
Dilution of red blood cells due to increased plasma volume
What is the characteristic of erythrocytosis?
Increased red blood cell count
What is the result of a shift of fluid from extracellular to intracellular compartments?
Dilution of red blood cells
What is the difference between relative anemia and true anemia?
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
What is the characteristic of relative erythrocytosis?
An apparent increase in red blood cell concentration due to decreased plasma volume
What is the characteristic of bone marrow in iron deficiency anemia?
Abundant iron stores in macrophages but not RBC precursors
What is the purpose of measuring sTfRs in serum in diagnosing iron deficiency anemia?
To distinguish iron deficiency from anemia of chronic inflammation
What is the significance of the Thomas plot in diagnosing iron deficiency anemia?
It distinguishes iron deficiency from anemia of chronic inflammation
What is the typical duration of iron replacement therapy?
6 months or longer
What happens to the products from the earlier stages in the pathway of heme synthesis when an enzyme is missing?
They accumulate in the cells
What is the characteristic of the porphyrias that have hematologic manifestations?
Only three of them have hematologic manifestations
What is the benefit of using oral bovine lactoferrin for iron supplementation?
It has reduced intestinal side effects compared to ferrous sulfate
In which cases is intravenous administration of iron dextrans used?
In rare cases with impaired intestinal absorption of iron
What is the consequence of the deposition of accumulated products in the skin?
Photosensitivity
What is the timeframe for reticulocyte counts to begin to increase after optimal iron treatment?
5-10 days
What is a common side effect of iron supplementation that can lead to poor patient compliance?
All of the above
Why is RBC transfusion rarely warranted for the correction of uncomplicated iron deficiency?
Because of the risks associated with RBC transfusions
What is the primary cause of chronic inflammation-related anemia?
Impaired ferrokinetics
How do inflammatory cytokines affect erythroid progenitor cells?
They decrease proliferation and responsiveness to erythropoietin
What is the effect of high hepcidin on macrophages and hepatocytes?
They cannot export iron to erythroblasts
What is the role of lactoferrin in iron acquisition by erythroblasts?
Erythroblasts cannot acquire iron from lactoferrin
What is the effect of chronic inflammation on RBC life span?
It decreases RBC life span
What is the role of hemophagocytic macrophages in chronic inflammation-related anemia?
They suppress erythropoiesis
What is the normal role of HFE in regulating iron absorption?
To reduce iron absorption by triggering hepcidin production
What is the effect of ferrous iron accumulation in cells?
It leads to the formation of reactive oxygen species
What is the consequence of mutated HFE not binding to B2-microglobulin?
It leads to increased iron absorption
What is the role of transferrin receptor 1 in the regulation of iron absorption?
It binds to HFE to trigger hepcidin production
What is the result of excess iron in parenchymal cells?
It leads to the formation of ferritin and hemosiderin
What is the normal function of HFE in the cell?
To bind to B2-microglobulin to regulate iron absorption
What is the purpose of lab testing in hemochromatosis?
All of the above
What may be elevated in individuals with hereditary hemochromatosis?
Serum ferritin and alanine transaminase level
What is essential to determine the degree of organ damage in hemochromatosis?
Liver biopsy and assessment of iron staining
Why may individuals with undiagnosed hereditary hemochromatosis come to medical attention?
Due to organ function problems leading to specific physical complaints
What can be used to monitor treatment in hemochromatosis?
Hemoglobin concentration and hematocrit
What can be used as a screening test for hereditary hemochromatosis?
Elevations of transferrin saturation or serum ferritin
This quiz covers the characteristics of Pelger-Huët anomaly, a rare genetic disorder affecting neutrophil nuclei, and its distinction from pseudo-PHA. Test your knowledge of PHA vs pseudo-PHA, cell features, and chromatin patterns.
Make Your Own Quizzes and Flashcards
Convert your notes into interactive study material.
Get started for free
