Chapter 33 Flashcards

1
Q

What is the average life span of a normal red blood cell in the circulation?

A

120 days

Normal red blood cells survive about 120 days before being removed by the spleen.

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2
Q

Which organ is most responsible for releasing erythropoietin (EPO) in response to hypoxia?

A

Kidney

The kidney’s interstitial fibroblasts are the primary source of erythropoietin.

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3
Q

What is the shape of mature red blood cells?

A

Biconcave discs without nuclei

RBCs lack nuclei and most organelles, maximizing surface area for gas exchange.

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4
Q

What is the primary function of red blood cells?

A

Transport oxygen and carbon dioxide

RBCs carry oxygen from the lungs to tissues and facilitate carbon dioxide transport back.

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5
Q

Hemoglobin is primarily composed of?

A

Four globin chains and four heme groups

Adult hemoglobin consists of two alpha and two beta globin chains, each with a heme group.

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6
Q

Which form of hemoglobin binds oxygen most readily?

A

Oxyhemoglobin

Oxyhemoglobin is hemoglobin that is bound to oxygen.

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7
Q

Iron is absorbed primarily in which part of the gastrointestinal tract?

A

Duodenum

The duodenum is the primary site of iron absorption.

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8
Q

Which cell type eventually becomes a mature RBC in the peripheral blood?

A

Reticulocyte

Reticulocytes are immature RBCs that mature into erythrocytes within about 1-2 days.

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9
Q

Erythropoietin production is stimulated by?

A

Low oxygen levels

Hypoxia in the kidney stimulates erythropoietin secretion.

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10
Q

The most important regulator of RBC production is?

A

Erythropoietin

Erythropoietin is the key hormone regulating RBC production in the bone marrow.

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11
Q

Which nutrient deficiency leads to megaloblastic anemia?

A

Vitamin B12 and folate

Deficiencies in vitamin B12 or folic acid impair DNA synthesis, causing megaloblastic anemia.

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12
Q

What is the role of transferrin in iron metabolism?

A

Transport of iron in the blood

Transferrin binds and transports iron through the bloodstream to tissues.

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13
Q

The majority of iron in the body is found in?

A

Hemoglobin in RBCs

About two-thirds of the body’s iron is in the heme structure of hemoglobin.

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14
Q

A low reticulocyte count in a patient with anemia suggests?

A

Reduced RBC production

A low reticulocyte count indicates inadequate bone marrow response.

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15
Q

Iron deficiency anemia typically presents as?

A

Microcytic, hypochromic anemia

Iron deficiency leads to smaller RBCs that are paler due to reduced hemoglobin content.

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16
Q

Pernicious anemia is caused by a deficiency of?

A

Intrinsic factor leading to B12 malabsorption

Pernicious anemia results from autoimmune gastritis that reduces intrinsic factor.

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17
Q

The normal red blood cell count in men is approximately?

A

5 million/µL

Normal RBC count for men averages around 5.2 million/µL.

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18
Q

Bilirubin is formed from the breakdown of?

A

The heme portion of hemoglobin

Heme is degraded to biliverdin and then bilirubin when RBCs are destroyed.

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19
Q

Polycythemia vera is characterized by?

A

Increased RBC mass due to a myeloproliferative disorder

Polycythemia vera is a bone marrow disorder causing excessive RBC production.

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20
Q

In response to chronic hypoxia, the body compensates by?

A

Increasing RBC production

Chronic hypoxia stimulates the kidney to produce more EPO, leading to increased RBC production.

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21
Q

The key regulator of iron absorption from the gut is?

A

Hepcidin

Hepcidin regulates intestinal iron absorption and release from stores.

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22
Q

Aplastic anemia involves?

A

Failure of the bone marrow to produce RBCs

Aplastic anemia results from inadequate hematopoiesis in the bone marrow.

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23
Q

What does ferritin do?

A

Stores iron, doesn’t regulate absorption directly

Ferritin is a protein that serves as a storage form of iron in the body.

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24
Q

What is aplastic anemia?

A

Failure of the bone marrow to produce RBCs

Aplastic anemia results in insufficient production of all blood cell types, including red blood cells.

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25
Q

Which substance is crucial for DNA synthesis in RBC precursors?

A

Vitamin B12

Vitamin B12 deficiency can lead to megaloblastic anemia due to impaired RBC maturation.

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26
Q

The oxygen-carrying capacity of blood is directly proportional to:

A

The hemoglobin concentration

More hemoglobin means a greater capacity to carry oxygen in the blood.

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27
Q

Which condition leads to normocytic, normochromic anemia?

A

Acute blood loss

Acute hemorrhage initially presents as normocytic, normochromic anemia before compensatory changes occur.

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28
Q

Hemolytic anemias are characterized by:

A

Increased RBC destruction

Hemolytic anemia involves the premature destruction of RBCs, leading to elevated bilirubin levels.

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29
Q

Carbonic anhydrase in RBCs facilitates:

A

Conversion of CO2 and H2O into H2CO3

This reaction aids in the transport of carbon dioxide in the blood.

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30
Q

The normal hematocrit for a healthy adult male is approximately:

A

45%

Normal male hematocrit is around 40-50%, with 45% being a common value.

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31
Q

In iron deficiency anemia, serum ferritin levels are typically:

A

Low

Ferritin levels drop in iron deficiency as it reflects body iron stores.

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32
Q

Excessive RBC destruction often leads to:

A

Jaundice (increased bilirubin)

Hemolysis increases free bilirubin load in the body, which can cause jaundice.

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33
Q

Hemoglobin F (fetal hemoglobin) differs from adult hemoglobin (HbA) because it:

A

Has a higher affinity for oxygen

This higher affinity facilitates oxygen transfer from maternal to fetal blood.

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34
Q

The final step in heme synthesis occurs in the:

A

Mitochondria of RBC precursors

Heme synthesis begins in the mitochondria, moves to the cytosol, and ends back in the mitochondria.

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35
Q

Iron overload (hemochromatosis) can damage organs due to:

A

Iron-mediated oxidative stress

Excess iron can lead to the formation of free radicals, causing tissue damage.

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36
Q

The normal mean corpuscular volume (MCV) for RBCs is approximately:

A

80-100 fL

Normal MCV indicates the average size of RBCs; values outside this range suggest microcytosis or macrocytosis.

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37
Q

Chronic renal failure often leads to anemia because:

A

The kidneys produce inadequate EPO

Damaged kidneys do not produce sufficient erythropoietin (EPO), reducing RBC production.

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38
Q

A key characteristic of sickle cell anemia is:

A

RBCs that form sickle shapes under low-oxygen conditions

Sickle cell anemia is caused by abnormal hemoglobin that polymerizes under low oxygen tension.

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39
Q

Which of the following can cause secondary polycythemia?

A

High altitude living conditions

Chronic hypoxia from high altitude increases EPO production, leading to more RBCs.

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40
Q

The majority of RBC energy (ATP) is generated via:

A

Glycolysis in the cytoplasm

RBCs lack mitochondria and rely on anaerobic glycolysis for ATP.

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41
Q

Which protein in RBC membranes maintains their biconcave shape?

A

Spectrin

Spectrin is a cytoskeletal protein essential for RBC membrane elasticity and shape.

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42
Q

In the absence of adequate iron, the RBCs formed will have:

A

Reduced hemoglobin content

Iron is required for hemoglobin synthesis; without it, RBCs are hypochromic and microcytic.

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43
Q

When RBCs are destroyed, the iron released from hemoglobin is:

A

Stored as ferritin or hemosiderin for reuse

The body efficiently recycles iron, storing it for future hemoglobin synthesis.

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44
Q

The primary stimulus for increased RBC production in secondary polycythemia is:

A

Low partial pressure of oxygen in tissues

Chronic hypoxia triggers increased EPO secretion, leading to more RBCs.

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45
Q

In macrocytic anemias, RBCs are larger because of:

A

Impaired DNA synthesis leading to delayed nuclear maturation

Macrocytosis arises from deficiencies in folate or B12, causing slow nuclear maturation.

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46
Q

The term ‘hematopoiesis’ refers to:

A

Production of blood cells

Hematopoiesis involves the formation and development of all blood cell types.

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47
Q

What does the term ‘hematopoiesis’ refer to?

A

Production of blood cells

Hematopoiesis means formation and development of all blood cell types.

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48
Q

In the early embryo, where are the first RBCs formed?

A

Yolk sac

Primitive erythropoiesis begins in the yolk sac during early embryonic life.

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49
Q

Which cells secrete the intrinsic factor necessary for vitamin B12 absorption?

A

Parietal cells of the stomach

Parietal cells secrete intrinsic factor, required for B12 absorption in the ileum.

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50
Q

Hematopoietic stem cells in the bone marrow give rise to which types of blood cells?

A

RBCs, WBCs, and platelets

Hematopoietic stem cells are multipotent and can differentiate into all blood cell lineages.

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51
Q

Why can’t RBCs repair themselves?

A

They have no DNA or nucleus

Mature RBCs lack a nucleus and organelles, preventing protein synthesis or damage repair.

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52
Q

Which condition results in RBCs that are abnormally fragile and prone to rupture?

A

Hereditary spherocytosis

Caused by defects in RBC cytoskeleton proteins, leading to spherical, fragile RBCs.

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53
Q

A patient with high RBC count and low EPO levels most likely has what condition?

A

Polycythemia vera

A primary polycythemia where RBCs proliferate independently, often with low EPO due to negative feedback.

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54
Q

Which glycolytic intermediate regulates oxygen release from hemoglobin?

A

2,3-Bisphosphoglycerate (2,3-BPG)

2,3-BPG binds to deoxyhemoglobin, stabilizing it and facilitating oxygen release to tissues.

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55
Q

Which RBC membrane protein is crucial for anchoring the spectrin-actin cytoskeleton?

A

Ankyrin

Ankyrin links spectrin to band 3 proteins in the RBC membrane, maintaining RBC shape and deformability.

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56
Q

Which index measures the variation in RBC size?

A

RDW (Red Cell Distribution Width)

RDW indicates the variation in RBC size; a high RDW suggests a wide range of cell sizes.

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57
Q

What is the earliest recognizable precursor of the RBC lineage in the bone marrow?

A

Proerythroblast

The proerythroblast is the first morphologically identifiable RBC precursor in the marrow.

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58
Q

What does poikilocytosis refer to?

A

Variation in RBC shape

Poikilocytosis is abnormal variation in RBC shape.

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59
Q

During early fetal life, the primary site of hematopoiesis transitions from the yolk sac to the:

A

Liver

After initial hematopoiesis in the yolk sac, the fetal liver becomes the main site of RBC production.

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60
Q

Which RBC enzyme pathway provides NADPH to maintain reduced glutathione?

A

Hexose monophosphate (pentose phosphate) shunt

This pathway generates NADPH to keep glutathione reduced, protecting RBCs from oxidative damage.

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61
Q

What triggers macrophages in the spleen to phagocytose aged RBCs?

A

Changes in RBC membrane carbohydrates and exposure of phosphatidylserine

Aging RBCs show altered membrane patterns, signaling splenic macrophages for removal.

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62
Q

How do RBCs assist in acid-base balance?

A

Carbonic anhydrase converting CO2 to bicarbonate and H+

This reaction facilitates CO2 transport and buffering in the blood.

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63
Q

Relative polycythemia can occur due to:

A

Dehydration reducing plasma volume

This results in an apparent increase in RBC count due to decreased plasma volume.

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64
Q

Which RBC index reflects the concentration of hemoglobin in a given volume of RBCs?

A

MCHC (Mean Corpuscular Hemoglobin Concentration)

MCHC measures the concentration of hemoglobin per unit volume of RBCs.

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65
Q

One of the key characteristics of extravascular hemolysis is:

A

RBC destruction primarily by macrophages in the spleen and liver

Extravascular hemolysis occurs within macrophages, not free in the bloodstream.

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66
Q

In intravascular hemolysis, free hemoglobin released into blood is bound by:

A

Haptoglobin

Haptoglobin binds free hemoglobin in plasma, preventing kidney damage and iron loss.

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67
Q

Lead poisoning affects RBCs by inhibiting which process?

A

Heme synthesis enzymes, leading to basophilic stippling

Lead inhibits enzymes like ferrochelatase and ALA dehydratase, causing abnormal RBCs.

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68
Q

The reticulocyte production index (RPI) adjusts the reticulocyte count for:

A

Degree of anemia and RBC lifespan

RPI corrects the reticulocyte percentage to account for the severity of anemia.

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69
Q

Which abnormal RBC form is characteristically seen in hereditary spherocytosis?

A

Spherocytes

Hereditary spherocytosis is due to membrane skeletal protein defects leading to spherical RBCs.

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70
Q

What causes hereditary spherocytosis?

A

Defects in membrane skeletal proteins leading to spherical RBCs

Hereditary spherocytosis results in a characteristic shape change in red blood cells.

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71
Q

What happens to stored RBCs in blood banks over time?

A

Decreased ATP and 2,3-BPG levels, reducing O2 delivery capacity

Stored RBCs lose energy and their ability to efficiently release oxygen.

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72
Q

An elevated RDW (Red Cell Distribution Width) is commonly associated with what?

A

A mixed population of microcytic and normocytic RBCs

High RDW indicates variability in red blood cell sizes, often seen during treatment for iron deficiency.

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73
Q

A high MCHC (Mean Corpuscular Hemoglobin Concentration) is most commonly seen in which condition?

A

Hereditary spherocytosis

Higher MCHC occurs due to the spherical shape and reduced volume of RBCs.

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74
Q

The presence of target cells (codocytes) is associated with which conditions?

A

Liver disease, thalassemia, and hemoglobinopathies

Target cells can be seen in various conditions affecting hemoglobin and cell morphology.

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75
Q

Basophilic stippling of RBCs is commonly observed in what?

A

Lead poisoning and thalassemia

This stippling indicates impaired maturation of red blood cells.

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76
Q

What is the key effect of increasing RBC 2,3-BPG levels at high altitude?

A

Increased oxygen delivery to tissues

Elevated 2,3-BPG reduces hemoglobin’s affinity for oxygen, promoting better oxygen release.

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77
Q

In the embryo, the very first RBCs formed in the yolk sac are characterized by what?

A

Primitive nucleated erythrocytes with embryonic hemoglobins

These early RBCs are different from adult hemoglobins and are involved in early oxygen transport.

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78
Q

RBC deformability is critical for what function?

A

Allowing RBCs to pass through narrow capillaries

Deformability is essential for effective oxygen delivery in the microcirculation.

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79
Q

When RBCs are destroyed intravascularly, what is a possible clinical sign?

A

Hemoglobinuria and hemosiderinuria

Free hemoglobin released into plasma and urine indicates intravascular hemolysis.

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80
Q

Which condition is characterized by defective synthesis of globin chains leading to microcytosis?

A

Thalassemia

Thalassemia results in microcytic anemia due to impaired globin chain production.

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81
Q

Iron overload can lead to what physiological response?

A

Increased hepcidin levels to limit iron absorption

Hepcidin regulates iron homeostasis, especially during overload conditions.

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82
Q

RBC aging is often marked by a decrease in what?

A

Enzyme activity and ATP levels

Aging RBCs lose functionality and signaling for removal as energy levels drop.

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83
Q

Which organ primarily monitors and removes aged RBCs from circulation?

A

Spleen (red pulp macrophages)

The spleen plays a crucial role in filtering and phagocytosing senescent red blood cells.

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84
Q

In anemia of chronic disease, increased hepcidin traps iron in storage sites, which reduces what?

A

Availability for RBC production

This mechanism contributes to anemia by limiting iron necessary for erythropoiesis.

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85
Q

A ‘shift to the right’ in the oxygen-hemoglobin dissociation curve is facilitated by what?

A

RBCs release 2,3-BPG, decreasing Hb’s oxygen affinity

This shift enhances oxygen release to tissues, especially under hypoxic conditions.

86
Q

In sickle cell disease, the mutation in the beta-globin chain replaces which amino acid?

A

Glutamic acid with valine

This specific mutation leads to the sickling of red blood cells under low oxygen conditions.

87
Q

In sideroblastic anemia, ringed sideroblasts form because?

A

Iron cannot be incorporated into heme properly

This defect leads to abnormal erythroblast maturation and iron accumulation.

88
Q

RBC lifespan in circulation is maintained despite no nucleus by what?

A

Stable enzyme systems and membrane maintenance pathways

RBCs rely on long-lived enzymes for functionality throughout their lifespan.

89
Q

The pentose phosphate pathway in RBCs mainly serves to produce what?

A

NADPH to maintain reduced glutathione

This function protects RBCs from oxidative damage.

90
Q

Erythropoiesis-stimulating agents (ESAs) can cause what?

A

Increased RBC mass and possibly elevated blood viscosity

ESAs stimulate RBC production, which may lead to increased hematocrit.

91
Q

What is the normal adult hemoglobin (HbA) composition?

A

α2β2

This composition is essential for normal oxygen transport in the blood.

92
Q

In anemia of chronic disease, iron is sequestered in storage sites due to what?

A

Increased hepcidin levels

Hepcidin’s role is crucial in regulating iron availability for erythropoiesis.

93
Q

In anemia of chronic disease, iron is sequestered in storage sites due to?

A

Increased hepcidin levels

Hepcidin blocks iron release from macrophages and enterocytes, leading to sequestration.

94
Q

In polycythemia vera, the mutation often found is in?

A

The JAK2 tyrosine kinase

Polycythemia vera frequently involves a JAK2 mutation, leading to erythrocytosis independent of EPO.

95
Q

RBC fragility tests measure?

A

The strength of RBC membranes under hypotonic conditions

Osmotic fragility tests assess RBC membrane stability in hypotonic solutions.

96
Q

RBC sedimentation rate (ESR) increases when?

A

Plasma fibrinogen and globulins increase, reducing RBC repulsion

Increased fibrinogen and other acute-phase reactants cause RBCs to stack (rouleaux), increasing ESR.

97
Q

The RBC’s unique biconcave shape helps by?

A

Increasing surface-to-volume ratio for efficient gas exchange

The biconcave shape increases surface area relative to volume, aiding rapid gas diffusion.

98
Q

Carbon monoxide (CO) interferes with RBC function by?

A

Displacing O2 from hemoglobin with higher affinity

CO binds hemoglobin with much greater affinity than O2, impairing O2 delivery.

99
Q

A decrease in plasma volume (as in dehydration) without a change in RBC mass results in?

A

Relative polycythemia

Reduced plasma volume elevates hematocrit, appearing as polycythemia but RBC mass is unchanged.

100
Q

The body’s inability to excrete excess iron easily leads to?

A

Iron loading in tissues (hemochromatosis)

With no regulated excretion route, excess iron accumulates, causing tissue damage.

101
Q

The hormone EPO acts on which cells in the bone marrow?

A

Progenitor and precursor cells in the erythroid lineage

EPO stimulates erythroid progenitors (CFU-E) and precursors to proliferate and differentiate.

102
Q

In G6PD deficiency, RBCs are more susceptible to oxidative stress because?

A

They have reduced glutathione levels due to less NADPH

G6PD deficiency impairs the pentose phosphate pathway, reducing NADPH and thus glutathione, increasing oxidative damage risk.

103
Q

In anemia of chronic disease, serum iron is often low because?

A

Hepcidin blocks iron release from macrophages and intestinal cells

Hepcidin sequesters iron in storage sites, reducing serum iron despite adequate stores.

104
Q

The lifespan of stored RBCs after transfusion is?

A

Often reduced due to storage lesion-related damage

Storage lesions cause reduced RBC deformability and shortened post-transfusion survival.

105
Q

Effective erythropoiesis ensures that?

A

Iron, vitamin B12, and folate availability match RBC production needs

Successful erythropoiesis depends on adequate supplies of iron, B12, and folate, as well as appropriate hormone regulation.

106
Q

What is the average life span of a normal red blood cell in the circulation?

A

120 days

Normal red blood cells survive about 120 days before being removed by the spleen.

107
Q

Which organ is most responsible for releasing erythropoietin (EPO) in response to hypoxia?

A

Kidney

The kidney’s interstitial fibroblasts are the primary source of erythropoietin.

108
Q

What is the shape of mature red blood cells?

A

Biconcave discs without nuclei

RBCs lack nuclei and most organelles, maximizing surface area for gas exchange.

109
Q

What is the primary function of red blood cells?

A

Transport oxygen and carbon dioxide

RBCs carry oxygen from the lungs to tissues and facilitate carbon dioxide transport back.

110
Q

Hemoglobin is primarily composed of?

A

Four globin chains and four heme groups

Adult hemoglobin consists of two alpha and two beta globin chains, each with a heme group.

111
Q

Which form of hemoglobin binds oxygen most readily?

A

Oxyhemoglobin

Oxyhemoglobin is hemoglobin that is bound to oxygen.

112
Q

Iron is absorbed primarily in which part of the gastrointestinal tract?

A

Duodenum

The duodenum is the primary site of iron absorption.

113
Q

Which cell type eventually becomes a mature RBC in the peripheral blood?

A

Reticulocyte

Reticulocytes are immature RBCs that mature into erythrocytes within about 1-2 days.

114
Q

Erythropoietin production is stimulated by?

A

Low oxygen levels

Hypoxia in the kidney stimulates erythropoietin secretion.

115
Q

The most important regulator of RBC production is?

A

Erythropoietin

Erythropoietin is the key hormone regulating RBC production in the bone marrow.

116
Q

Which nutrient deficiency leads to megaloblastic anemia?

A

Vitamin B12 and folate

Deficiencies in vitamin B12 or folic acid impair DNA synthesis, causing megaloblastic anemia.

117
Q

What is the role of transferrin in iron metabolism?

A

Transport of iron in the blood

Transferrin binds and transports iron through the bloodstream to tissues.

118
Q

The majority of iron in the body is found in?

A

Hemoglobin in RBCs

About two-thirds of the body’s iron is in the heme structure of hemoglobin.

119
Q

A low reticulocyte count in a patient with anemia suggests?

A

Reduced RBC production

A low reticulocyte count indicates inadequate bone marrow response.

120
Q

Iron deficiency anemia typically presents as?

A

Microcytic, hypochromic anemia

Iron deficiency leads to smaller RBCs that are paler due to reduced hemoglobin content.

121
Q

Pernicious anemia is caused by a deficiency of?

A

Intrinsic factor leading to B12 malabsorption

Pernicious anemia results from autoimmune gastritis that reduces intrinsic factor.

122
Q

The normal red blood cell count in men is approximately?

A

5 million/µL

Normal RBC count for men averages around 5.2 million/µL.

123
Q

Bilirubin is formed from the breakdown of?

A

The heme portion of hemoglobin

Heme is degraded to biliverdin and then bilirubin when RBCs are destroyed.

124
Q

Polycythemia vera is characterized by?

A

Increased RBC mass due to a myeloproliferative disorder

Polycythemia vera is a bone marrow disorder causing excessive RBC production.

125
Q

In response to chronic hypoxia, the body compensates by?

A

Increasing RBC production

Chronic hypoxia stimulates the kidney to produce more EPO, leading to increased RBC production.

126
Q

The key regulator of iron absorption from the gut is?

A

Hepcidin

Hepcidin regulates intestinal iron absorption and release from stores.

127
Q

Aplastic anemia involves?

A

Failure of the bone marrow to produce RBCs

Aplastic anemia results from inadequate hematopoiesis in the bone marrow.

128
Q

What does ferritin do?

A

Stores iron, doesn’t regulate absorption directly

Ferritin is a protein that serves as a storage form of iron in the body.

129
Q

What is aplastic anemia?

A

Failure of the bone marrow to produce RBCs

Aplastic anemia results in insufficient production of all blood cell types, including red blood cells.

130
Q

Which substance is crucial for DNA synthesis in RBC precursors?

A

Vitamin B12

Vitamin B12 deficiency can lead to megaloblastic anemia due to impaired RBC maturation.

131
Q

The oxygen-carrying capacity of blood is directly proportional to:

A

The hemoglobin concentration

More hemoglobin means a greater capacity to carry oxygen in the blood.

132
Q

Which condition leads to normocytic, normochromic anemia?

A

Acute blood loss

Acute hemorrhage initially presents as normocytic, normochromic anemia before compensatory changes occur.

133
Q

Hemolytic anemias are characterized by:

A

Increased RBC destruction

Hemolytic anemia involves the premature destruction of RBCs, leading to elevated bilirubin levels.

134
Q

Carbonic anhydrase in RBCs facilitates:

A

Conversion of CO2 and H2O into H2CO3

This reaction aids in the transport of carbon dioxide in the blood.

135
Q

The normal hematocrit for a healthy adult male is approximately:

A

45%

Normal male hematocrit is around 40-50%, with 45% being a common value.

136
Q

In iron deficiency anemia, serum ferritin levels are typically:

A

Low

Ferritin levels drop in iron deficiency as it reflects body iron stores.

137
Q

Excessive RBC destruction often leads to:

A

Jaundice (increased bilirubin)

Hemolysis increases free bilirubin load in the body, which can cause jaundice.

138
Q

Hemoglobin F (fetal hemoglobin) differs from adult hemoglobin (HbA) because it:

A

Has a higher affinity for oxygen

This higher affinity facilitates oxygen transfer from maternal to fetal blood.

139
Q

The final step in heme synthesis occurs in the:

A

Mitochondria of RBC precursors

Heme synthesis begins in the mitochondria, moves to the cytosol, and ends back in the mitochondria.

140
Q

Iron overload (hemochromatosis) can damage organs due to:

A

Iron-mediated oxidative stress

Excess iron can lead to the formation of free radicals, causing tissue damage.

141
Q

The normal mean corpuscular volume (MCV) for RBCs is approximately:

A

80-100 fL

Normal MCV indicates the average size of RBCs; values outside this range suggest microcytosis or macrocytosis.

142
Q

Chronic renal failure often leads to anemia because:

A

The kidneys produce inadequate EPO

Damaged kidneys do not produce sufficient erythropoietin (EPO), reducing RBC production.

143
Q

A key characteristic of sickle cell anemia is:

A

RBCs that form sickle shapes under low-oxygen conditions

Sickle cell anemia is caused by abnormal hemoglobin that polymerizes under low oxygen tension.

144
Q

Which of the following can cause secondary polycythemia?

A

High altitude living conditions

Chronic hypoxia from high altitude increases EPO production, leading to more RBCs.

145
Q

The majority of RBC energy (ATP) is generated via:

A

Glycolysis in the cytoplasm

RBCs lack mitochondria and rely on anaerobic glycolysis for ATP.

146
Q

Which protein in RBC membranes maintains their biconcave shape?

A

Spectrin

Spectrin is a cytoskeletal protein essential for RBC membrane elasticity and shape.

147
Q

In the absence of adequate iron, the RBCs formed will have:

A

Reduced hemoglobin content

Iron is required for hemoglobin synthesis; without it, RBCs are hypochromic and microcytic.

148
Q

When RBCs are destroyed, the iron released from hemoglobin is:

A

Stored as ferritin or hemosiderin for reuse

The body efficiently recycles iron, storing it for future hemoglobin synthesis.

149
Q

The primary stimulus for increased RBC production in secondary polycythemia is:

A

Low partial pressure of oxygen in tissues

Chronic hypoxia triggers increased EPO secretion, leading to more RBCs.

150
Q

In macrocytic anemias, RBCs are larger because of:

A

Impaired DNA synthesis leading to delayed nuclear maturation

Macrocytosis arises from deficiencies in folate or B12, causing slow nuclear maturation.

151
Q

The term ‘hematopoiesis’ refers to:

A

Production of blood cells

Hematopoiesis involves the formation and development of all blood cell types.

152
Q

What does the term ‘hematopoiesis’ refer to?

A

Production of blood cells

Hematopoiesis means formation and development of all blood cell types.

153
Q

In the early embryo, where are the first RBCs formed?

A

Yolk sac

Primitive erythropoiesis begins in the yolk sac during early embryonic life.

154
Q

Which cells secrete the intrinsic factor necessary for vitamin B12 absorption?

A

Parietal cells of the stomach

Parietal cells secrete intrinsic factor, required for B12 absorption in the ileum.

155
Q

Hematopoietic stem cells in the bone marrow give rise to which types of blood cells?

A

RBCs, WBCs, and platelets

Hematopoietic stem cells are multipotent and can differentiate into all blood cell lineages.

156
Q

Why can’t RBCs repair themselves?

A

They have no DNA or nucleus

Mature RBCs lack a nucleus and organelles, preventing protein synthesis or damage repair.

157
Q

Which condition results in RBCs that are abnormally fragile and prone to rupture?

A

Hereditary spherocytosis

Caused by defects in RBC cytoskeleton proteins, leading to spherical, fragile RBCs.

158
Q

A patient with high RBC count and low EPO levels most likely has what condition?

A

Polycythemia vera

A primary polycythemia where RBCs proliferate independently, often with low EPO due to negative feedback.

159
Q

Which glycolytic intermediate regulates oxygen release from hemoglobin?

A

2,3-Bisphosphoglycerate (2,3-BPG)

2,3-BPG binds to deoxyhemoglobin, stabilizing it and facilitating oxygen release to tissues.

160
Q

Which RBC membrane protein is crucial for anchoring the spectrin-actin cytoskeleton?

A

Ankyrin

Ankyrin links spectrin to band 3 proteins in the RBC membrane, maintaining RBC shape and deformability.

161
Q

Which index measures the variation in RBC size?

A

RDW (Red Cell Distribution Width)

RDW indicates the variation in RBC size; a high RDW suggests a wide range of cell sizes.

162
Q

What is the earliest recognizable precursor of the RBC lineage in the bone marrow?

A

Proerythroblast

The proerythroblast is the first morphologically identifiable RBC precursor in the marrow.

163
Q

What does poikilocytosis refer to?

A

Variation in RBC shape

Poikilocytosis is abnormal variation in RBC shape.

164
Q

During early fetal life, the primary site of hematopoiesis transitions from the yolk sac to the:

A

Liver

After initial hematopoiesis in the yolk sac, the fetal liver becomes the main site of RBC production.

165
Q

Which RBC enzyme pathway provides NADPH to maintain reduced glutathione?

A

Hexose monophosphate (pentose phosphate) shunt

This pathway generates NADPH to keep glutathione reduced, protecting RBCs from oxidative damage.

166
Q

What triggers macrophages in the spleen to phagocytose aged RBCs?

A

Changes in RBC membrane carbohydrates and exposure of phosphatidylserine

Aging RBCs show altered membrane patterns, signaling splenic macrophages for removal.

167
Q

How do RBCs assist in acid-base balance?

A

Carbonic anhydrase converting CO2 to bicarbonate and H+

This reaction facilitates CO2 transport and buffering in the blood.

168
Q

Relative polycythemia can occur due to:

A

Dehydration reducing plasma volume

This results in an apparent increase in RBC count due to decreased plasma volume.

169
Q

Which RBC index reflects the concentration of hemoglobin in a given volume of RBCs?

A

MCHC (Mean Corpuscular Hemoglobin Concentration)

MCHC measures the concentration of hemoglobin per unit volume of RBCs.

170
Q

One of the key characteristics of extravascular hemolysis is:

A

RBC destruction primarily by macrophages in the spleen and liver

Extravascular hemolysis occurs within macrophages, not free in the bloodstream.

171
Q

In intravascular hemolysis, free hemoglobin released into blood is bound by:

A

Haptoglobin

Haptoglobin binds free hemoglobin in plasma, preventing kidney damage and iron loss.

172
Q

Lead poisoning affects RBCs by inhibiting which process?

A

Heme synthesis enzymes, leading to basophilic stippling

Lead inhibits enzymes like ferrochelatase and ALA dehydratase, causing abnormal RBCs.

173
Q

The reticulocyte production index (RPI) adjusts the reticulocyte count for:

A

Degree of anemia and RBC lifespan

RPI corrects the reticulocyte percentage to account for the severity of anemia.

174
Q

Which abnormal RBC form is characteristically seen in hereditary spherocytosis?

A

Spherocytes

Hereditary spherocytosis is due to membrane skeletal protein defects leading to spherical RBCs.

175
Q

What causes hereditary spherocytosis?

A

Defects in membrane skeletal proteins leading to spherical RBCs

Hereditary spherocytosis results in a characteristic shape change in red blood cells.

176
Q

What happens to stored RBCs in blood banks over time?

A

Decreased ATP and 2,3-BPG levels, reducing O2 delivery capacity

Stored RBCs lose energy and their ability to efficiently release oxygen.

177
Q

An elevated RDW (Red Cell Distribution Width) is commonly associated with what?

A

A mixed population of microcytic and normocytic RBCs

High RDW indicates variability in red blood cell sizes, often seen during treatment for iron deficiency.

178
Q

A high MCHC (Mean Corpuscular Hemoglobin Concentration) is most commonly seen in which condition?

A

Hereditary spherocytosis

Higher MCHC occurs due to the spherical shape and reduced volume of RBCs.

179
Q

The presence of target cells (codocytes) is associated with which conditions?

A

Liver disease, thalassemia, and hemoglobinopathies

Target cells can be seen in various conditions affecting hemoglobin and cell morphology.

180
Q

Basophilic stippling of RBCs is commonly observed in what?

A

Lead poisoning and thalassemia

This stippling indicates impaired maturation of red blood cells.

181
Q

What is the key effect of increasing RBC 2,3-BPG levels at high altitude?

A

Increased oxygen delivery to tissues

Elevated 2,3-BPG reduces hemoglobin’s affinity for oxygen, promoting better oxygen release.

182
Q

In the embryo, the very first RBCs formed in the yolk sac are characterized by what?

A

Primitive nucleated erythrocytes with embryonic hemoglobins

These early RBCs are different from adult hemoglobins and are involved in early oxygen transport.

183
Q

RBC deformability is critical for what function?

A

Allowing RBCs to pass through narrow capillaries

Deformability is essential for effective oxygen delivery in the microcirculation.

184
Q

When RBCs are destroyed intravascularly, what is a possible clinical sign?

A

Hemoglobinuria and hemosiderinuria

Free hemoglobin released into plasma and urine indicates intravascular hemolysis.

185
Q

Which condition is characterized by defective synthesis of globin chains leading to microcytosis?

A

Thalassemia

Thalassemia results in microcytic anemia due to impaired globin chain production.

186
Q

Iron overload can lead to what physiological response?

A

Increased hepcidin levels to limit iron absorption

Hepcidin regulates iron homeostasis, especially during overload conditions.

187
Q

RBC aging is often marked by a decrease in what?

A

Enzyme activity and ATP levels

Aging RBCs lose functionality and signaling for removal as energy levels drop.

188
Q

Which organ primarily monitors and removes aged RBCs from circulation?

A

Spleen (red pulp macrophages)

The spleen plays a crucial role in filtering and phagocytosing senescent red blood cells.

189
Q

In anemia of chronic disease, increased hepcidin traps iron in storage sites, which reduces what?

A

Availability for RBC production

This mechanism contributes to anemia by limiting iron necessary for erythropoiesis.

190
Q

A ‘shift to the right’ in the oxygen-hemoglobin dissociation curve is facilitated by what?

A

RBCs release 2,3-BPG, decreasing Hb’s oxygen affinity

This shift enhances oxygen release to tissues, especially under hypoxic conditions.

191
Q

In sickle cell disease, the mutation in the beta-globin chain replaces which amino acid?

A

Glutamic acid with valine

This specific mutation leads to the sickling of red blood cells under low oxygen conditions.

192
Q

In sideroblastic anemia, ringed sideroblasts form because?

A

Iron cannot be incorporated into heme properly

This defect leads to abnormal erythroblast maturation and iron accumulation.

193
Q

RBC lifespan in circulation is maintained despite no nucleus by what?

A

Stable enzyme systems and membrane maintenance pathways

RBCs rely on long-lived enzymes for functionality throughout their lifespan.

194
Q

The pentose phosphate pathway in RBCs mainly serves to produce what?

A

NADPH to maintain reduced glutathione

This function protects RBCs from oxidative damage.

195
Q

Erythropoiesis-stimulating agents (ESAs) can cause what?

A

Increased RBC mass and possibly elevated blood viscosity

ESAs stimulate RBC production, which may lead to increased hematocrit.

196
Q

What is the normal adult hemoglobin (HbA) composition?

A

α2β2

This composition is essential for normal oxygen transport in the blood.

197
Q

In anemia of chronic disease, iron is sequestered in storage sites due to what?

A

Increased hepcidin levels

Hepcidin’s role is crucial in regulating iron availability for erythropoiesis.

198
Q

In anemia of chronic disease, iron is sequestered in storage sites due to?

A

Increased hepcidin levels

Hepcidin blocks iron release from macrophages and enterocytes, leading to sequestration.

199
Q

In polycythemia vera, the mutation often found is in?

A

The JAK2 tyrosine kinase

Polycythemia vera frequently involves a JAK2 mutation, leading to erythrocytosis independent of EPO.

200
Q

RBC fragility tests measure?

A

The strength of RBC membranes under hypotonic conditions

Osmotic fragility tests assess RBC membrane stability in hypotonic solutions.

201
Q

RBC sedimentation rate (ESR) increases when?

A

Plasma fibrinogen and globulins increase, reducing RBC repulsion

Increased fibrinogen and other acute-phase reactants cause RBCs to stack (rouleaux), increasing ESR.

202
Q

The RBC’s unique biconcave shape helps by?

A

Increasing surface-to-volume ratio for efficient gas exchange

The biconcave shape increases surface area relative to volume, aiding rapid gas diffusion.

203
Q

Carbon monoxide (CO) interferes with RBC function by?

A

Displacing O2 from hemoglobin with higher affinity

CO binds hemoglobin with much greater affinity than O2, impairing O2 delivery.

204
Q

A decrease in plasma volume (as in dehydration) without a change in RBC mass results in?

A

Relative polycythemia

Reduced plasma volume elevates hematocrit, appearing as polycythemia but RBC mass is unchanged.

205
Q

The body’s inability to excrete excess iron easily leads to?

A

Iron loading in tissues (hemochromatosis)

With no regulated excretion route, excess iron accumulates, causing tissue damage.

206
Q

The hormone EPO acts on which cells in the bone marrow?

A

Progenitor and precursor cells in the erythroid lineage

EPO stimulates erythroid progenitors (CFU-E) and precursors to proliferate and differentiate.

207
Q

In G6PD deficiency, RBCs are more susceptible to oxidative stress because?

A

They have reduced glutathione levels due to less NADPH

G6PD deficiency impairs the pentose phosphate pathway, reducing NADPH and thus glutathione, increasing oxidative damage risk.

208
Q

In anemia of chronic disease, serum iron is often low because?

A

Hepcidin blocks iron release from macrophages and intestinal cells

Hepcidin sequesters iron in storage sites, reducing serum iron despite adequate stores.

209
Q

The lifespan of stored RBCs after transfusion is?

A

Often reduced due to storage lesion-related damage

Storage lesions cause reduced RBC deformability and shortened post-transfusion survival.

210
Q

Effective erythropoiesis ensures that?

A

Iron, vitamin B12, and folate availability match RBC production needs

Successful erythropoiesis depends on adequate supplies of iron, B12, and folate, as well as appropriate hormone regulation.