Pelger-Huët Anomaly Quiz

Play an AI-generated podcast conversation about this lesson

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?

Pink or red stained needle-like crystals (C) Signup and view all the answers

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

Faggot cells (C) Signup and view all the answers

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

63-93% (D) Signup and view all the answers

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

Mutation in the MYH9 gene affecting megakaryocyte maturation (C) Signup and view all the answers

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

They are larger and rounder in shape (B) Signup and view all the answers

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

Mitochondrial myopathies (A) Signup and view all the answers

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

40-80 x 10^9/L (B) Signup and view all the answers

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

Myosin Heavy Chain Type IIA (C) Signup and view all the answers

What is the underlying defect in Lysosomal Storage Diseases?

Flawed degradation of phagocytized material (A) Signup and view all the answers

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

70% (A) Signup and view all the answers

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

Decreased ability of phagocytes to produce superoxide and reactive oxygen species (C) Signup and view all the answers

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 (D) Signup and view all the answers

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

To form hydrogen peroxide with the addition of water (A) Signup and view all the answers

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

NITROBLUE TETRAZOLIUM REDUCTION TEST (A) Signup and view all the answers

What is the characteristic finding in Lazy Leukocyte Syndrome?

Neutropenia (A) Signup and view all the answers

What is the primary defect in Schwachman Syndrome?

Defective actin filaments in neutrophils (B) Signup and view all the answers

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

To compensate for decreased oxygen in the air (B) Signup and view all the answers

What is the consequence of defective NADPH oxidase in neutrophils?

Decreased ability of phagocytes to produce superoxide and reactive oxygen species (D) Signup and view all the answers

What is the characteristic of catalase-positive microorganisms?

They are able to break down H2O2 (A) Signup and view all the answers

What is the primary functionally definition of anemia?

A decrease in the oxygen carrying capacity of the blood (A) Signup and view all the answers

What is the result of a normal Nitroblue Tetrazolium test?

The yellow substance is reduced to a dark blue insoluble formazan (C) Signup and view all the answers

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

It indicates a hematologic disorder (C) Signup and view all the answers

What is the characteristic of moderate anemia?

Hemoglobin concentration of 7-10 g/dL (C) Signup and view all the answers

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

Decreased ability of phagocytes to kill engulfed microorganisms (A) Signup and view all the answers

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

X-linked, but some autosomal recessive or point mutations (D) Signup and view all the answers

What is the characteristic of megaloblastic anemia?

Oval macrocyte, large oval RBC (A) Signup and view all the answers

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

It indicates increased RBC destruction (D) Signup and view all the answers

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 (D) Signup and view all the answers

What is the characteristic of erythrocytosis?

Increased red blood cell count (C) Signup and view all the answers

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

Dilution of red blood cells (A) Signup and view all the answers

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 (C) Signup and view all the answers

What is the characteristic of relative erythrocytosis?

An apparent increase in red blood cell concentration due to decreased plasma volume (A) Signup and view all the answers

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

Abundant iron stores in macrophages but not RBC precursors (C) Signup and view all the answers

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

To distinguish iron deficiency from anemia of chronic inflammation (D) Signup and view all the answers

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

It distinguishes iron deficiency from anemia of chronic inflammation (B) Signup and view all the answers

What is the typical duration of iron replacement therapy?

6 months or longer (C) 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?

They accumulate in the cells (C) Signup and view all the answers

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

Only three of them have hematologic manifestations (B) Signup and view all the answers

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

It has reduced intestinal side effects compared to ferrous sulfate (B) Signup and view all the answers

In which cases is intravenous administration of iron dextrans used?

In rare cases with impaired intestinal absorption of iron (C) Signup and view all the answers

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

Photosensitivity (A) Signup and view all the answers

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

5-10 days (A) Signup and view all the answers

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

All of the above (D) Signup and view all the answers

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

Because of the risks associated with RBC transfusions (A) Signup and view all the answers

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

Impaired ferrokinetics (C) Signup and view all the answers

How do inflammatory cytokines affect erythroid progenitor cells?

They decrease proliferation and responsiveness to erythropoietin (A) Signup and view all the answers

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

They cannot export iron to erythroblasts (C) Signup and view all the answers

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

Erythroblasts cannot acquire iron from lactoferrin (D) Signup and view all the answers

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

It decreases RBC life span (A) Signup and view all the answers

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

They suppress erythropoiesis (A) Signup and view all the answers

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

To reduce iron absorption by triggering hepcidin production (A) Signup and view all the answers

What is the effect of ferrous iron accumulation in cells?

It leads to the formation of reactive oxygen species (C) Signup and view all the answers

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

It leads to increased iron absorption (A) Signup and view all the answers

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

It binds to HFE to trigger hepcidin production (B) Signup and view all the answers

What is the result of excess iron in parenchymal cells?

It leads to the formation of ferritin and hemosiderin (B) Signup and view all the answers

What is the normal function of HFE in the cell?

To bind to B2-microglobulin to regulate iron absorption (D) Signup and view all the answers

What is the purpose of lab testing in hemochromatosis?