Erythrocytes Flashcards

1
Q

Manual RBC count is rarely done because of its

A

inaccuracy and questionable necessity

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2
Q

other more accurate manual rbc tests when automation is unavailable

A

microhematocrit
hemoglobin concentration

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3
Q

T/F

Manual WBC count and PLT count are now unreliable.

A

False

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4
Q

People living in higher altitude have higher

A

RBC count, hematocrit, hemoglobin

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5
Q

At 2 km altitude, hgb is elevated at around

A

1 g Hb/dL

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6
Q

At 3 km altitude, Hgb will be elevated at around

A

2 g Hb/dL

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7
Q

RBC count reference range for children (8-13 yo) and adult women

A

conventional: 4.00-5.40 x 10^6/uL
SI: 4.00-5.40 x 10^12/uL

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8
Q

RBC count reference range for adult male

A

conventional: 4.60-6.00 x 10^6/uL
SI: 4.60 - 6.00 x 10^12/uL

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9
Q

Major glycolytic pathway

A

Embden-Meyerhof Pathway

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10
Q

Glycolysis diversion pathways or alternate pathways that branch from the glycolytic pathway

A

Hexose monophosphate shunt
Methemoglobin reductase shunt
Rapaport-Luebering shunt

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11
Q

Glucose penetrates the rbc with no energy expenditure via this transmembrane protein

A

Glut-1

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12
Q

EMP handles what percent of glycolization in the RBCs

A

90%

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13
Q

EMP handles what percent of glycolization in the RBCs

A

90%

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14
Q

T/F

EMP is a non-oxidative, anaerobic pathway

A

True

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15
Q

How many molecules of ATP is produced by EMP

A

2 molecules of ATP

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16
Q

ATP is used by the RBC in 3 ways:

A

maintenance of shape and deformability
gives energy for active transport of cations
helps modulate the amount 2,3-BPG

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17
Q

EMP modulates the amount of 2,3-BPG, while _______ actually creates 2,3-DPG

A

Rapaport-Luebering Shunt

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18
Q

2,3-BPG meaning

A

2,3-Bisphosphoglycerate

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19
Q

2,3-BPG was previously known as

A

2,3-Diphosphoglycerate (2,3-DPG)

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20
Q

To what particular pathway is the Pyruvate kinase deficiency related to?

A

Embden-Meyerhof Pathway

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21
Q

HNSHA stands for

A

Hereditary nonspherocytic hemolytic anemia

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22
Q

most common enzyme deficiency of the EMP and is the most common form of HNSHA

A

PK deficiency

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23
Q

Possible PBS findings in PK deficiency

A

acanthocytes
burr cells

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24
Q

recommended screening test for PK deficiency

A

PK fluorescent spot test

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25
Q

Screening tests for PK deficiency

A

PK fluorescent spot test
Autohemolysis test

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26
Q

confirmatory test for PK deficiency

A

Quantitative PK assay

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27
Q

In PK deficiency Type II, autohemolysis is greatly increased and glucose has no effect. What molecule corrects the hemolysis?

A

ATP

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28
Q

Hexose monophosphate shunt is also known as

A

Pentose Phosphate Pathway
Phosphogluconate Pathway

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29
Q

This shunt aerobically converts glucose to pentose and produces reduced nicotinamide adenine dinucleotide phosphate

A

PPP/HMP/PGP

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30
Q

NADPH reduces

A

glutathione

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31
Q

Reduced glutathione reduces peroxides and guards proteins, lipids, and heme iron from

A

oxidation

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32
Q

HMP is functionally dependent on

A

G6PD

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33
Q

What patway is G6PD related to?

A

HMP

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34
Q

Most common enzyme deficiency in the PPP and is the most common RBC enzyme defect

A

G6PD deficiency

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35
Q

prevalence of G6PD deficiency

A

5% of the world population
about 400 million people worldwide

36
Q

Possible PBS findings in G6PD deficiency include

A

heinz bodies
bite cells

37
Q

Possible PBS findings in G6PD deficiency include

A

heinz bodies
bite cells

38
Q

screening tests for G6PD deficiency

A

G6PD fluorescent spot test
autohemolysis test

39
Q

recommended screening test for G6PD deficiency

A

G6PD fluorescent spot test

40
Q

confirmatory test for G6PD deficiency

A

Quantitative G6PD assay

41
Q

Pattern of hemolysis in G6PD deficiency

A

autohemolysis is slightly/moderately increased but is partially corrected by glucose

42
Q

Inclusion bodies brought about by the precipitation of the globin portion of the hemoglobin due to the absence of G6PD.

A

Heinz bodies

43
Q

Heinz bodies undergo _______ in the spleen

A

pitting

44
Q

The process of removing a part of the rbc

A

Pitting

45
Q

After heinz bodies are removed by splenic macrophages during pitting, the rbc becomes

A

bite cells

46
Q

Bite cells undergo _______ in the spleen

A

culling

47
Q

The process of removing a whole rbc in the circulation

A

culling

48
Q

Repeated culling will lead to

A

hemolytic anemia

49
Q

G6PD deficiency is classified by the WHO into

A

5 classes

50
Q

G6PD-Madrid and G6PD-Serres belong in what class

A

Class I

51
Q

G6PD-Madrid and G6PD-Serres belong in what class

A

Class I

52
Q

Class I G6PD-deficiency have severely deficient enzyme activity. State the %

A

<1% enzymatic activity or not detectable

53
Q

clinical manifestations of class I G6PD-deficiency

A

chronic HNSHA, variable severity, rare

54
Q

examples of Class II G6PD-deficiency

A

G6PD-Chatham
G6PD-Mediterranean

55
Q

G6PD enzyme activity in class II G6PD-deficiency

A

severely deficient, <10% activity

56
Q

class II G6PD-deficiency has severe episodic acute hemolytic anemia associated with infections, certain drugs, and ______

A

fava beans

57
Q

T/F

Class II G6PD-deficiency is self-limited and does not require blood transfusions during hemolytic episodes

A

False

58
Q

Only a small group of G6PD-deficient px demonstrate unusual sensitivity to fava beans and most of these people have what variant of G6PD-deficiency?

A

G6PD-Mediterannean variant

59
Q

unusual sensitivity to fava beans

A

favism

60
Q

Class III G6PD-deficiency is mild to moderately deficient with _____ enzyme activity

A

10-60%

61
Q

T/F

Class III G6PD-deficiency is not self-limited and may require blood transfusions during hemolytic episodes that are associated with infections and certain drugs,

A

False

62
Q

Variants of Class III G6PD-deficiency

A

G6PD-A^-
G6PD-Canton

63
Q

what classes of G6PD-deficiency have no clinical manifestations

A

Class IV and V

64
Q

Class IV G6PD-deficiency has mildly deficient to normal enzymatic activity, with _____ % activity

A

60-150%

65
Q

variants of class IV G6PD-deficiency

A

G6PD-B (wildtype)
G6PD-A^+

66
Q

G6PD-A^+ may also manifest as

A

Class III G6PD-deficiency

67
Q

Class V G6PD-deficiency has increased enzymatic activity:

A

> 150% activity

68
Q

Some foods/chemicals to avoid if px has G6PD-deficiency

A

legumes
mothballs
red wine
fava beans
blueberry
bitter gourd
menthol
soya food

69
Q

Methemoglobin reductase is also known as

A

cytochrome b5 reductase

70
Q

methemoglobin reductase pathway maintains iron in its

A

reduced form (ferrous)

71
Q

methemoglobin reductase pathway maintains iron in its

A

reduced form (ferrous)

72
Q

The pathway that protects the globin portion of the hemoglobin

A

HMS

73
Q

Pathway that protects the heme portion of hemoglobin effectively

A

MRP

74
Q

Pathway that protects the heme portion of hemoglobin effectively

A

MRP

75
Q

T/F

HMS can also protect the heme from being oxidized. However, it is less effective than MRP in doing so.

A

True

76
Q

Rapoport-Luebering Shunt produces

A

2,3-BPG

77
Q

Binds to Hgb and decreases the affinity of O2 to Hgb

A

2,3-BPG

78
Q

two variables that affect the degree of association/dissociation between oxygen and hemoglobin

A

pO2
affinity of Hgb for O2

79
Q

the affinity of hemoglobin for oxygen is dependent on 5 factors

A

pH
pCO2
2,3-BPG
Temperature
presence of other Hgb species that are non-functional

80
Q

what happens to the affinity of oxygen to hgb if pH is increased

A

increased affinity

81
Q

what happens to the affinity of oxygen to hgb if pH is decreased

A

affinity is decreased

82
Q

the curve produced when po2 and the affinity of hgb for O2 are plotted on a graph

A

oxygen dissociation curve

83
Q

increased pH
decreased pCO2, 2,3-BPG, and temp

A

shift to the left

84
Q

decreased pH
increased pCO2, 2,3-BPG, and temp

A

shift to the right

85
Q

a shift in the curve due to an alteration in pH or the effect of H ions and CO2 on the affinity of hemoglobin for O2

A

Bohr effect

86
Q

Depicts the occurrence by which the binding of O2 to the hemoglobin promotes the release of CO2

A

Haldane effect