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Biochem 42

front 1

A skin biopsy 6 hours after bacterial cellulitis shows the earliest leukocyte influx expected in acute inflammation. Which cell type should predominate first?

A) Basophils

B) Monocytes

C) Eosinophils

D) Neutrophils

back 1

D. Neutrophils

front 2

A child with helminth infection and asthma has increased leukocytes with large red cytoplasmic granules. Which association best matches this cell?

A) Viral killing; lymph nodes

B) Allergy; parasites; chronic inflammation

C) Acute bacteria; pus formation

D) Antibody production; plasma cells

back 2

B. Allergy; parasites; chronic inflammation

front 3

Which statement best matches eosinophils with their classic microscopic and tissue features?

A) Red granules

B) Blue granules

C) No granules

D) Tertiary granules

back 3

A. Red granules

front 4

A healthy adult female has blood centrifuged for hematocrit measurement. Which packed red-cell volume is expected?

A) 20–30%

B) 25–35%

C) 35–45%

D) 50–60%

back 4

C. 35–45%

front 5

Which hemoglobin-chain pairing correctly describes adult hemoglobin A?

A) Two alpha; two beta

B) Two alpha; two gamma

C) Two alpha; two delta

D) Two beta; two delta

back 5

A. Two alpha; two beta

front 6

Which hemoglobin-chain pairing correctly describes adult hemoglobin A2?

A) Two beta; two gamma

B) Two alpha; two delta

C) Two alpha; two beta

D) Two gamma; two delta

back 6

B. Two alpha; two delta

front 7

Which hemoglobin-chain pairing correctly describes fetal hemoglobin?

A) Two alpha; two beta

B) Two beta; two delta

C) Two alpha; two delta

D) Two alpha; two gamma

back 7

D. Two alpha; two gamma

front 8

A patient with sickle cell disease has abnormal polymerizing hemoglobin. Which mutation best explains the disease?

A) Alpha-chain deletion

B) Gamma-chain persistence

C) Beta-globin point mutation

D) Delta-chain duplication

back 8

C. Beta-globin point mutation

front 9

In sickle cell anemia, which amino-acid substitution occurs in the abnormal beta-globin chain?

A) Valine to glutamic acid

B) Glutamic acid to valine

C) Lysine to arginine

D) Glycine to proline

back 9

B. Glutamic acid to valine

front 10

Which hemoglobin mutation name is classically present in sickle cell anemia?

A) HbC

B) HbA2

C) HbF

D) HbS

back 10

D. HbS

front 11

During hematopoiesis, which progenitor gives rise to erythroid colony-forming units?

A) Multipotential myeloid stem cell

B) Mature erythrocyte

C) Circulating reticulocyte

D) Megakaryocyte lineage

back 11

A. Multipotential myeloid stem cell

front 12

Which erythropoiesis transition occurs earliest in normal red-cell maturation?

A) Reticulocyte to erythrocyte

B) Normoblast to reticulocyte

C) Proerythroblast to basophilic erythroblast

D) Band cell to neutrophil

back 12

C. Proerythroblast to basophilic erythroblast

front 13

Which late erythropoiesis sequence is correctly ordered?

A) Reticulocyte before erythrocyte

B) Erythrocyte before normoblast

C) Myelocyte before reticulocyte

D) Band before erythrocyte

back 13

A. Reticulocyte before erythrocyte

front 14

A patient living at high altitude develops increased red-cell production. Which organ-hormone pairing drives this response?

A) Liver; thrombopoietin

B) Spleen; histamine

C) Marrow; PDGF

D) Kidney; erythropoietin

back 14

D. Kidney; erythropoietin

front 15

A patient with chronic blood loss develops small pale red cells. Which deficiency best matches this microcytic anemia?

A) Folate deficiency

B) Iron deficiency

C) Vitamin B12 deficiency

D) EPO deficiency

back 15

B. Iron deficiency

front 16

A patient with impaired DNA synthesis develops enlarged red-cell precursors. Which deficiency best matches macrocytic anemia?

A) Folate deficiency

B) Iron deficiency

C) Histamine deficiency

D) PDGF deficiency

back 16

A. Folate deficiency

front 17

Which classification correctly divides white blood cells into two major groups?

A) Platelets and erythrocytes

B) Myeloid and erythroid cells

C) Granulocytes and agranulocytes

D) Plasma cells and stem cells

back 17

C. Granulocytes and agranulocytes

front 18

Which set contains only granulocytes?

A) Monocytes; lymphocytes; NK cells

B) Basophils; eosinophils; neutrophils

C) Platelets; monocytes; neutrophils

D) B cells; T cells; basophils

back 18

B. Basophils; eosinophils; neutrophils

front 19

Among granulocytes, which cell type is classically described as having tertiary granules?

A) Neutrophils

B) Basophils

C) Eosinophils

D) Monocytes

back 19

A. Neutrophils

front 20

After leaving marrow, neutrophils can occupy which two peripheral compartments?

A) Formation; storage

B) Circulating; marginating

C) Erythroid; megakaryocytic

D) B-cell; T-cell

back 20

B. Circulating; marginating

front 21

A neutrophil tethers to vascular endothelium during inflammation. Which adhesion molecule is involved in this binding?

A) E-selectin

B) P-selectin

C) Integrin beta-chain

D) L-selectin

back 21

D. L-selectin

front 22

Which cell is the first recognizable precursor in granulocyte development?

A) Myeloblast

B) Promyelocyte

C) Metamyelocyte

D) Band cell

back 22

A. Myeloblast

front 23

Which granulocyte maturation sequence is correctly ordered after the myeloblast stage?

A) Myelocyte; promyelocyte; band; metamyelocyte

B) Promyelocyte; myelocyte; metamyelocyte; band

C) Band; metamyelocyte; myelocyte; promyelocyte

D) Metamyelocyte; band; promyelocyte; myelocyte

back 23

B. Promyelocyte; myelocyte; metamyelocyte; band

front 24

Which two leukocyte classes are agranulocytes?

A) Neutrophils and basophils

B) Eosinophils and monocytes

C) Lymphocytes and monocytes

D) Platelets and lymphocytes

back 24

C. Lymphocytes and monocytes

front 25

The bipotential CFU-GM progenitor can differentiate into which two lineages?

A) Platelets or erythrocytes

B) Monocytes or neutrophils

C) B cells or T cells

D) Eosinophils or basophils

back 25

B. Monocytes or neutrophils

front 26

Which marrow-derived platelet precursor normally remains in bone marrow rather than circulating intact?

A) Megakaryocyte

B) Reticulocyte

C) Myeloblast

D) Normoblast

back 26

A. Megakaryocyte

front 27

Which list correctly names the four platelet granule types?

A) Primary; secondary; tertiary; azurophilic

B) Alpha; beta; gamma; delta

C) Dense; clear; red; blue

D) Alpha; delta; gamma; lambda

back 27

D. Alpha; delta; gamma; lambda

front 28

Which major lymphocyte types should be grouped together?

A) B cells; T cells; NK cells

B) Monocytes; neutrophils; NK cells

C) Basophils; eosinophils; B cells

D) Platelets; T cells; monocytes

back 28

A. B cells; T cells; NK cells

front 29

A patient undergoes splenic immune activation after a bloodstream infection. Which set best summarizes normal spleen functions?

A) Store bile; activate neutrophils

B) Make EPO; filter lymph

C) Activate lymphocytes; filter blood antigens

D) Produce platelets; degrade heme

back 29

C. Activate lymphocytes; filter blood antigens

front 30

A patient with chronic hypoxemia develops increased erythropoiesis. Which kidney-derived hormone mediates this response?

A) Erythropoietin

B) Thrombopoietin

C) Angiotensin II

D) Aldosterone

back 30

A. Erythropoietin

front 31

In mature erythrocytes, which metabolic route is dominant and feeds the pathway producing 2,3-BPG?

A) TCA cycle; malate shunt

B) Beta-oxidation; carnitine shuttle

C) Pentose pathway; NADPH route

D) Glycolysis; Rapoport-Leubering shunt

back 31

D. Glycolysis; Rapoport-Leubering shunt

front 32

The hexose monophosphate shunt protects RBCs from oxidative damage by generating which paired products?

A) ATP; oxidized glutathione

B) NADPH; reduced glutathione

C) FADH2; reduced hemoglobin

D) NADH; ferric iron

back 32

B. NADPH; reduced glutathione

front 33

What is involved in the first and what is involved in the rate-limiting step in heme biosynthesis:

A) Acetyl-CoA and alanine

B) Citrate and serine

C) Succinyl-CoA and glycine

D) Pyruvate and glutamate

back 33

C. Succinyl-CoA and glycine

front 34

A blood smear shows malformed spherical RBCs, while another sample shows immature circulating RBCs. Which pairing is correct?

A) Spherocytes; reticulocytes

B) Schistocytes; normoblasts

C) Reticulocytes; spherocytes

D) Megakaryocytes; platelets

back 34

A. Spherocytes; reticulocytes

front 35

A marrow biopsy shows large multinucleated cells releasing cytoplasmic fragments into sinusoids. Which blood element is produced this way?

A) Eosinophils

B) Monocytes

C) Reticulocytes

D) Platelets

back 35

D. Platelets

front 36

A patient has microcytic, hypochromic anemia due impaired hemoglobin synthesis. Which cause set best fits?

A) B12; folate; erythroleukemia

B) Iron def; thalassemia; lead poisoning

C) Bleeding; sickle disease; PK defect

D) Splenectomy; malaria; methemoglobinemia

back 36

B. Iron def; thalassemia; lead poisoning

front 37

A patient develops macrocytic, normochromic anemia from impaired DNA synthesis. Which cause set best fits?

A) Iron deficiency; lead poisoning

B) Acute bleeding; sickle disease

C) B12 deficiency; folate deficiency

D) G6PD deficiency; malaria resistance

back 37

C. B12 deficiency; folate deficiency

front 38

A patient has normocytic, normochromic anemia from red-cell loss or destruction. Which cause set best fits?

A) Bleeding; sickle disease; metabolic defects

B) Iron deficiency; thalassemia; lead poisoning

C) B12 deficiency; folate deficiency

D) DMT1 mutation; pyridoxine deficiency

back 38

A. Bleeding; sickle disease; metabolic defects

front 39

A patient at high altitude has increased erythrocyte 2,3-BPG. What is the main physiologic effect?

A) Increased hemoglobin oxygen affinity

B) Blocked oxygen release to tissues

C) Methemoglobin reduction in RBCs

D) Enhanced oxygen unloading to tissues

back 39

D. Enhanced oxygen unloading to tissues

remember that 2,3 bpg helps unload o2 onto tissues

front 40

An inherited RBC enzyme deficiency causes hemolytic anemia, with compensatory increased 2,3-BPG improving tissue oxygen delivery. Which enzyme is deficient?

A) Cytochrome b5 reductase

B) Glucose-6-phosphatase

C) Pyruvate kinase

D) Ferrochelatase

back 40

C. Pyruvate kinase

front 41

Congenital methemoglobinemia can result from hemoglobin M or deficiency of which enzyme?

A) Cytochrome b5 reductase

B) Glucose-6-P dehydrogenase

C) Gamma-ALA synthase

D) Pyruvate kinase

back 41

A. Cytochrome b5 reductase

front 42

Which inherited RBC enzyme deficiency is common in humans partly because it protects against malaria?

A) Pyruvate kinase deficiency

B) G6PD deficiency

C) Cytochrome b5 reductase deficiency

D) DMT1 deficiency

back 42

B. G6PD deficiency

front 43

A patient with G6PD-deficient red cells is being evaluated for RBC survival. Which change is expected?

A) Longer RBC lifespan

B) Normal RBC half-life

C) Increased platelet survival

D) Reduced RBC half-life

back 43

D. Reduced RBC half-life

front 44

A malnourished patient develops microcytic, hypochromic anemia because the first heme-synthesis reaction slows. Which deficiency best explains this?

A) Vitamin B12 deficiency

B) Folate deficiency

C) Pyridoxine deficiency

D) Vitamin C deficiency

back 44

C. Pyridoxine deficiency

front 45

A child with abdominal pain and microcytic anemia has accumulation of gamma-ALA and protoporphyrin IX. Which exposure best explains this?

A) Lead

B) Iron

C) Phenobarbital

D) Malaria

back 45

A. Lead

front 46

Dietary iron is absorbed as Fe2+ but must become Fe3+ for transport. Which protein performs this oxidation?

A) Ferritin

B) Hemosiderin

C) Transferrin

D) Ceruloplasmin

back 46

D. Ceruloplasmin

front 47

Which iron-handling pairing is correct for blood transport, usual storage, and excess storage?

A) Ferritin; transferrin; DMT1

B) Transferrin; ferritin; hemosiderin

C) Hemosiderin; ferritin; transferrin

D) Ceruloplasmin; DMT1; transferrin

back 47

B. Transferrin; ferritin; hemosiderin

front 48

A patient has photosensitivity, facial hypertrichosis, and scarring due an inherited heme-synthesis enzyme defect. Which disorder group fits best?

A) Porphyrias

B) Thalassemias

C) Sideroblastic anemias

D) Methemoglobinemias

back 48

A. Porphyrias

front 49

Which variable best correlates with the lifetime of an erythrocyte?

A) Plasma transferrin saturation

B) Splenic platelet storage

C) Glucose-6-P activity

D) Serum ceruloplasmin activity

back 49

C. Glucose-6-P activity

front 50

A patient has microcytic, hypochromic anemia due an inherited SLC11A2 mutation. Which encoded protein is affected?

A) Ferroportin

B) DMT-1

C) Transferrin

D) Ceruloplasmin

back 50

B. DMT-1

front 51

delete

back 51

delete

front 52

A physician evaluating iron deficiency anemia in an adult man worries about occult GI blood loss. Which etiologies best match this concern?

A) Ulcers or colon cancer

B) Asthma or eczema

C) Hemophilia or leukemia

D) Malaria or porphyria

back 52

A. Ulcers or colon cancer

front 53

Phenobarbital induces cytochrome P450 enzymes that use heme. Which compensatory response increases heme synthesis?

A) Reduced DMT1 expression

B) Induced gamma-ALA synthase

C) Blocked bilirubin conjugation

D) Increased cytochrome b5 reductase

back 53

B. Induced gamma-ALA synthase

front 54

During normal heme catabolism, which product is conjugated with glucuronic acid and excreted in bile?

A) Protoporphyrin IX

B) Gamma-ALA

C) Bilirubin

D) Hemosiderin

back 54

C. Bilirubin

front 55

An RBC membrane disorder affects the major erythrocyte cytoskeletal protein. Which protein is most likely defective?

A) Ferritin

B) Transferrin

C) Ceruloplasmin

D) Spectrin

back 55

D. Spectrin

front 56

A patient has hemolytic anemia from unstable erythrocyte membranes. Which defect category best explains this?

A) Cytoskeletal protein defects

B) Platelet granule defects

C) EPO receptor deactivation

D) Transferrin transport defects

back 56

A. Cytoskeletal protein defects

front 57

A bone marrow transplant sample is enriched before infusion to improve engraftment. Which cell population should be increased?

A) Reticulocytes

B) Stem cells

C) Mature erythrocytes

D) Megakaryocytes

back 57

B. Stem cells

front 58

A family has excessive RBC production from an EPO receptor mutation causing sustained JAK2-STAT5 signaling. Which negative regulator cannot deactivate the receptor?

A) DMT-1

B) Spectrin

C) SHP-1

D) Ceruloplasmin

back 58

C. SHP-1

front 59

Which mechanism best explains megaloblastic anemia from folate or B12 deficiency?

A) Early nuclear extrusion after fewer divisions

B) Excess hemoglobin concentration per cell

C) Increased splenic platelet sequestration

D) Ferric hemoglobin accumulation

back 59

A. Early nuclear extrusion after fewer divisions

front 60

HbC differs from normal beta-globin by which substitution at the same position affected in HbS?

A) Glu-to-Val

B) Lys-to-Glu

C) Val-to-Glu

D) Glu-to-Lys

back 60

D. Glu-to-Lys

"C" for crystal, sounds like lysine i guess

front 61

Why are HbS/HbC patients usually more symptomatic than HbA/HbS patients?

A) Less HbS polymerizes in RBCs

B) Higher HbS concentration enhances sickling

C) HbC prevents oxygen unloading

D) HbA increases RBC dehydration

back 61

B. Higher HbS concentration enhances sickling

front 62

Which disorder group results from imbalanced alpha- and beta-globin chain synthesis?

A) Porphyrias

B) Methemoglobinemias

C) Thalassemias

D) Spherocytoses

back 62

C. Thalassemias

front 63

Absence of all four alpha-globin genes is classically associated with which condition?

A) Hydrops fetalis

B) HbC disease

C) Beta-thalassemia minor

D) Congenital methemoglobinemia

back 63

A. Hydrops fetalis

front 64

In beta-thalassemia, which genotype is expected to cause severe disease?

A) β+/β+

B) βA/βS

C) β+/βA

D) β0/β0

back 64

D. β0/β0

front 65

Compared with HbA, HbF binds 2,3-BPG differently in a way that causes which effect?

A) Lower 2,3-BPG affinity; higher oxygen affinity

B) Higher 2,3-BPG affinity; lower oxygen affinity

C) Lower 2,3-BPG affinity; lower oxygen affinity

D) Higher 2,3-BPG affinity; higher oxygen affinity

back 65

A. Lower 2,3-BPG affinity; higher oxygen affinity

front 66

Patients with beta-thalassemia or sickle cell anemia often have milder disease when which hemoglobin is elevated?

A) HbA

B) HbA2

C) HbF

D) HbC

back 66

C. HbF

front 67

Mutations in spectrin, ankyrin, or band 3 most directly cause which RBC abnormality?

A) Methemoglobin formation

B) Spherocyte formation

C) HbF persistence

D) Porphyrin accumulation

back 67

B. Spherocyte formation

front 68

Which pairing correctly describes the alpha-globin locus?

A) Chromosome 11; epsilon gene

B) Chromosome 11; gamma genes

C) Chromosome 16; zeta and alpha genes

D) Chromosome 16; beta and delta genes

back 68

C. Chromosome 16; zeta and alpha genes

front 69

Which pairing correctly describes the beta-globin locus?

A) Chromosome 11; epsilon and fetal genes

B) Chromosome 16; zeta and alpha genes

C) Chromosome 11; only beta genes

D) Chromosome 16; gamma and beta genes

back 69

A. Chromosome 11; epsilon and fetal genes

front 70

Normal functional hemoglobin within RBCs contains iron in which form?

A) Ferric

B) Ferrous

C) Hemosiderin-bound

D) Transferrin-bound

back 70

B. Ferrous

front 71

Ferric hemoglobin within RBCs is best described by which term?

A) Oxyhemoglobin

B) Carboxyhemoglobin

C) Deoxyhemoglobin

D) Methemoglobin

back 71

D. Methemoglobin

front 72

Inherited defects in heme-synthesis enzymes produce which disease group?

A) Thalassemias

B) Porphyrias

C) Spherocytoses

D) Hemoglobinopathies

back 72

B. Porphyrias

front 73

Unlike mature RBCs, white blood cells normally have which structural feature?

A) Nuclei

B) Hemoglobin

C) Spectrin

D) Reticulin

back 73

A. Nuclei

front 74

Which granulocyte rapidly migrates to infection or tissue damage?

A) Basophil

B) Eosinophil

C) Monocyte

D) Neutrophil

back 74

D. Neutrophil

front 75

Which cell type is especially important against parasites and removes fibrin during inflammation?

A) Eosinophil

B) Basophil

C) Neutrophil

D) Lymphocyte

back 75

A. Eosinophil

front 76

A patient with asthma and allergic disease has increased granulocytes. Which cell is most expected?

A) Neutrophils

B) Eosinophils

C) Monocytes

D) NK cells

back 76

B. Eosinophils

front 77

Increased eosinophils are associated with which condition set?

A) Asthma; allergies; autoimmune disease

B) Bleeding; hemolysis; iron deficiency

C) Thymic maturation; viral killing

D) Platelet budding; clot retraction

back 77

A. Asthma; allergies; autoimmune disease

front 78

Which granulocyte participates in hypersensitivity reactions and stores histamine?

A) Neutrophil

B) Eosinophil

C) Basophil

D) Monocyte

back 78

C. Basophil

front 79

A lymphocyte precursor must mature into a T cell. Which organ is required?

A) Thymus

B) Spleen

C) Bone marrow

D) Lymph node

back 79

A. Thymus

front 80

Which site is responsible for B-cell maturation?

A) Thymus

B) Bone marrow

C) Spleen

D) Liver

back 80

B. Bone marrow

front 81

Tissue macrophages arise from which circulating precursor cell?

A) Neutrophils

B) Basophils

C) Monocytes

D) Reticulocytes

back 81

C. Monocytes

front 82

An RBC enzyme defect blocks phosphoenolpyruvate conversion to pyruvate. Which metabolite is expected to rise?

A) Heme

B) 2,3-BPG

C) Methemoglobin

D) Reduced glutathione

back 82

B. 2,3-BPG

front 83

Erythrocyte glycolysis generates 2,3-BPG through which pathway?

A) Hexose monophosphate shunt

B) Urea cycle

C) Rapoport-Luebering shunt

D) Carnitine shuttle

back 83

C. Rapoport-Luebering shunt

front 84

How does 2,3-BPG promote oxygen delivery to tissues?

A) Stabilizes deoxyhemoglobin

B) Stabilizes ferric hemoglobin

C) Oxidizes ferrous iron

D) Activates cytochrome b5

back 84

A. Stabilizes deoxyhemoglobin

front 85

Congenital methemoglobinemia is classically caused by deficiency of which enzyme?

A) Ferrochelatase

B) G6PD

C) δ-ALA synthase

D) Cytochrome b5 reductase

back 85

D. Cytochrome b5 reductase

front 86

Inherited hemoglobin M causes methemoglobinemia by stabilizing iron in which state?

A) Fe0

B) Fe2+

C) Fe3+

D) Fe4+

back 86

C. Fe3+

front 87

For hemoglobin to bind oxygen normally, iron must be in which form?

A) Ferric; Fe3+

B) Ferrous; Fe2+

C) Heme-free; Fe0

D) Oxidized; Fe4+

back 87

B. Ferrous; Fe2+

front 88

Cytochrome b5 is regenerated in RBCs using which reducing agent?

A) NADH

B) NADPH

C) FADH2

D) ATP

back 88

A. NADH

front 89

Which RBC pathway generates NADPH for antioxidant protection?

A) Rapoport-Luebering shunt

B) TCA cycle

C) Hexose monophosphate shunt

D) Beta-oxidation

back 89

C. Hexose monophosphate shunt

front 90

The chief RBC defense against ROS injury to proteins and lipids is which molecule?

A) Ferritin

B) Reduced glutathione

C) Transferrin

D) Methemoglobin

back 90

B. Reduced glutathione

front 91

Which molecule maintains glutathione in its reduced protective state?

A) NADPH

B) NADH

C) 2,3-BPG

D) δ-ALA

back 91

A. NADPH

front 92

Which enzyme catalyzes the first step of the hexose monophosphate shunt?

A) δ-ALA dehydratase

B) G6PD

C) Ferrochelatase

D) Porphobilinogen deaminase

back 92

B. G6PD

front 93

Which porphyrin type is most common in nature?

A) Type I

B) Type II

C) Type III

D) Type IV

back 93

C. Type III

front 94

Which porphyrin is the most common in the human body?

A) Bilirubin

B) Heme

C) Porphobilinogen

D) Protoporphyrinogen

back 94

B. Heme

front 95

Known G6PD variant genes most often contain which mutation types?

A) Nonsense; frameshift deletions

B) Missense; in-frame deletions

C) Duplications; promoter insertions

D) Translocations; trinucleotide repeats

back 95

B. Missense; in-frame deletions

front 96

Heme synthesis begins with which two starter substrates?

A) Glycine; succinyl CoA

B) Alanine; acetyl CoA

C) Serine; pyruvate

D) Glutamate; citrate

back 96

A. Glycine; succinyl CoA

front 97

The first intermediate formed from glycine and succinyl CoA in heme synthesis is what?

A) Protoporphyrin IX

B) Porphobilinogen

C) δ-Aminolevulinic acid

D) Uroporphyrinogen III

back 97

C. δ-Aminolevulinic acid

front 98

Producing one heme molecule requires how many glycines and succinyl CoA molecules?

A) Two of each

B) Four of each

C) Six of each

D) Eight of each

back 98

D. Eight of each

front 99

Heme regulates its own synthesis by repressing which enzyme?

A) δ-ALA synthase

B) Ferrochelatase

C) Uroporphyrinogen decarboxylase

D) Coproporphyrinogen oxidase

back 99

A. δ-ALA synthase

front 100

δ-ALA dehydratase converts δ-ALA into which pyrrole product?

A) Porphobilinogen

B) Heme

C) Biliverdin

D) Protoporphyrin IX

back 100

A. Porphobilinogen

front 101

Lead poisoning directly inhibits which two heme-synthesis enzymes?

A) G6PD and ferrochelatase

B) δ-ALA dehydratase and ferrochelatase

C) Porphobilinogen deaminase and G6PD

D) Cytochrome b5 and DMT-1

back 101

B. δ-ALA dehydratase and ferrochelatase

front 102

A child with lead toxicity develops impaired heme synthesis. Which metabolites accumulate?

A) δ-ALA and protoporphyrin IX

B) Heme and bilirubin

C) Glycine and succinyl CoA

D) Ferritin and transferrin

back 102

A. δ-ALA and protoporphyrin IX

front 103

Which nutrient increases absorption of nonheme iron from the digestive tract?

A) Vitamin B12

B) Folate

C) Vitamin C

D) Vitamin K

back 103

C. Vitamin C

front 104

Which pairing correctly describes iron absorption and transport states?

A) Absorbed Fe3+; transported Fe2+

B) Absorbed Fe2+; transported Fe3+

C) Absorbed Fe0; transported Fe2+

D) Absorbed Fe3+; transported Fe0

back 104

B. Absorbed Fe2+; transported Fe3+

front 105

A patient with excess porphyrin production develops skin symptoms after sunlight exposure. Which symptom best fits?

A) Photosensitivity

B) Hemarthrosis

C) Night blindness

D) Splenomegaly

back 105

A. Photosensitivity

front 106

Iron is carried in blood when apotransferrin binds iron to form which complex?

A) Ferritin

B) Transferrin

C) Hemosiderin

D) Ceruloplasmin

back 106

B. Transferrin

front 107

Ferrous iron leaves the endosome and enters cytoplasm through which transporter?

A) Band 3

B) Ferrochelatase

C) DMT-1

D) Ankyrin

back 107

C. DMT-1

front 108

An SLC11A2 mutation disrupts DMT-1 function. Which anemia pattern is expected?

A) Macrocytic normochromic anemia

B) Iron-deficiency anemia

C) Aplastic anemia

D) Megaloblastic anemia

back 108

B. Iron-deficiency anemia

front 109

How does heme regulate hemoglobin synthesis?

A) Stimulates globin synthesis

B) Blocks transferrin binding

C) Degrades spectrin

D) Inhibits bilirubin conjugation

back 109

A. Stimulates globin synthesis

front 110

Normal heme degradation ultimately forms which pigment?

A) Stercobilin

B) Ferritin

C) Bilirubin

D) Protoporphyrin IX

back 110

C. Bilirubin

front 111

The first step of heme degradation releases which gaseous product?

A) CO

B) NO

C) O2

D) CO2

back 111

A. CO

front 112

In the liver, bilirubin reacts with UDP-glucuronate to form which water-soluble product?

A) Urobilinogen

B) Bilirubin monoglucuronide

C) Stercobilin

D) Protoporphyrin IX

back 112

B. Bilirubin monoglucuronide

front 113

Intestinal bacteria deconjugate bilirubin diglucuronide into which compound?

A) Urobilinogen

B) UDP-glucuronate

C) Biliverdin

D) Hemosiderin

back 113

A. Urobilinogen

front 114

Most intestinal urobilinogen is converted into which fecal pigment?

A) Bilirubin

B) Heme

C) Stercobilin

D) Ferritin

back 114

C. Stercobilin

front 115

Which organ determines whether circulating RBCs remain viable?

A) Liver

B) Kidney

C) Thymus

D) Spleen

back 115

D. Spleen

front 116

Which pairing correctly links spectrin to the RBC membrane bilayer?

A) Ankyrin; assisted by band 3

B) DMT-1; assisted by ferritin

C) Transferrin; assisted by ceruloplasmin

D) Actin; assisted by HbF

back 116

A. Ankyrin; assisted by band 3

front 117

Which RBC cytoskeletal protein helps stabilize ankyrin-band 3 membrane attachment?

A) Band 4.2

B) Glycophorin C

C) Ferrochelatase

D) Globin

back 117

A. Band 4.2

front 118

Band 4.1 anchors spectrin by binding which two proteins?

A) Ankyrin and band 3

B) Glycophorin C and actin

C) Ferritin and transferrin

D) DMT-1 and ceruloplasmin

back 118

B. Glycophorin C and actin

front 119

Leukemias are best described as which hematopoietic abnormality?

A) Excess platelet granule release

B) Complete erythrocyte maturation

C) Incomplete hematopoietic cell development

D) Accelerated bilirubin conjugation

back 119

C. Incomplete hematopoietic cell development

front 120

In sickle-cell anemia, which beta-globin substitution produces HbS?

A) E6K

B) G6V

C) E6V

D) V6E

back 120

C. E6V

front 121

HbC is caused by which beta-globin substitution?

A) E6V

B) E6K

C) K6E

D) V6K

back 121

B. E6K

front 122

Which feature is characteristic of HbC protein within RBCs?

A) Hemoglobin precipitation

B) Ferric iron stabilization

C) Increased HbF switching

D) Decreased globin synthesis

back 122

A. Hemoglobin precipitation

HbC disease causes abnormal hemoglobin that tends to precipitate/crystallize inside RBCs.

This makes RBCs less flexible and more likely to be removed by the spleen, causing mild hemolytic anemia.

front 123

An imbalance with excess production of one hemoglobin subunit over another causes which disease class?

A) Porphyrias

B) Methemoglobinemias

C) Thalassemias

D) Leukemias

back 123

C. Thalassemias

front 124

Alpha-thalassemias usually arise from which genetic defect?

A) β-promoter point mutations

B) Complete α-globin gene deletions

C) δ and β deletions

D) Missense ferrochelatase mutations

back 124

B. Complete α-globin gene deletions

front 125

Which thalassemia type is generally more severe?

A) α-thalassemia

B) β-thalassemia

C) δ-thalassemia

D) γ-thalassemia

back 125

B. β-thalassemia

front 126

Which β-thalassemia genotype ranking is most severe to least severe?

A) β+/β+ > β+/β0 > β0/β0

B) β0/β0 > β+/β0 > β+/β+

C) β+/β0 > β0/β0 > β+/β+

D) β+/β+ > β0/β0 > β+/β0

back 126

B. β0/β0 > β+/β0 > β+/β+

front 127

Excess β-chains in thalassemia can form which homotetramer

A) HbS

B) HbC

C) HbF

D) HbH

back 127

D. HbH

front 128

Excess α-chain production damages which RBC cytoskeletal protein?

A) Band 4.1

B) Ankyrin

C) DMT-1

D) Ceruloplasmin

back 128

A. Band 4.1