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Hema 1 > ERYTHROCYTES (Part 5) > Flashcards

ERYTHROCYTES (Part 5) Flashcards

1
Q

This is rarely done because of its inaccuracy and questionable necessity.

A

Manual RBC counts (Obsolete)

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2
Q

When automation is not available, what manual RBC tests are more accurate?

A

microhematocrit and hemoglobin concentration

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3
Q

Values that are ELEVATED in people living at a HIGHER ALTITUDE over what they would be at sea level?

A

RBC Count
Hemoglobin
Hematocrit

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4
Q

The difference in values in relation to the altitude?

A

1 g Hb/dL at 2 km altitude
2 g Hb/dL at 3 km altitude

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5
Q

Conventional unit for Children (8 to 13 y.o.):

4.00 to 5.40 x 10^6/μL

A

4.00 to 5.40 x 10^12/L

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6
Q

Conventional unit for Adult (male):

4.60 to 6.00 x 10^6/μL

A

4.60 to 6.00 x 10^12/L

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7
Q

Conventional unit for Adult (female):

4.00 to 5.40 x 10^6/μL

A

4.00 to 5.40 x 10^12/L

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8
Q

major glycolytic pathway

A

Embden-Meyerhof Pathway

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9
Q

Glucose penetrates the red blood cell with no energy expenditure via GLUT-1 (a transmembrane protein).

A

Embden-Meyerhof Pathway

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10
Q

Handles 90% of glucose utilization in the RBCs

A

Embden-Meyerhof Pathway

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11
Q

Non-oxidative, ANAEROBIC pathway that produces 2 MOLECULES of ATP

A

Embden-Meyerhof Pathway

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12
Q

ATP - used by the RBCs in the following ways:

A
  1. Maintenance of RBC shape & deformability
  2. Give energy for the active transport of cations
  3. Helps in modulating the amount o
    2,3 - Bisphosphoglycerate (2, 3 BFG)
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13
Q

Old name of 2,3 - Bisphosphoglycerate (2, 3 BFG)?

A

2, 3 - Diphosphoglycerate (2, 3 DPG)

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14
Q

Old RBC becomes more ____________.

A

spherocytic

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15
Q

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

A

Pyruvate kinase (PK) deficiency

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16
Q

Possible PBS findings or PK deficiency include?

A

Acanthocytes Burr calls

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17
Q

What is the meaning of HNSHA?

A

Hereditary Nonspherocytic Hemolytic Anemia

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18
Q

Recommended screening test for PK deficiency

A

PK fluorescent spot test

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19
Q

A screening test for PK deficiency

A

Autohemolysis test

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20
Q

Pattern of autohemolysis associated with PK deficiency?

A

Autohemolysis is greatly increased and glucose has no effect (but ATP corrects the hemolysis) [TYPE II].

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21
Q

Confirmatory test for PK deficiency?

A

Quantitative PK assay

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22
Q

Three alternate pathways that branch from the glycolytic pathway?

A
  1. Hexose Monophosphate Shunt (Aerobic Glycolysis)
  2. Methemoglobin Reductase Pathway/Shunt (MRP) (MRS)
  3. Rapoport-Luebering Shunt
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23
Q

Other names for Hexose Monophosphate Shunt [HMS] (Aerobic Glycolysis)?

A

Pentose Phosphate Pathway or Phosphogluconate Pathway

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24
Q

aerobically converts glucose to pentose and produces NADPH (reduced)

A

HMS

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25
Q

What is the meaning of NADPH?

A

Nicotinamide adenine dinucleotide phosphate

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26
Q

Can also protect the HEME IRON but less effective than the MRP/MRS?

A

HMS

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27
Q

Prevents the denaturation of the GLOBIN by oxidation?

A

HMS

28
Q

Functionally dependent on G6PD (important to HMS to operate normally)?

A

HMS

29
Q

NADPH reduces to?

A

GLUTATHIONE (reduced glutathione reduces peroxides and guards proteins, lipids, and heme iron from oxidation)

30
Q

Most common enzyme deficiency in the pentose phosphate pathway?

A

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

31
Q

MOST COMMON RBC enzyme defect (prevalence of 5% of the global population, or approximately 400 million people worldwide)?

A

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

32
Q

Possible PBS findings of G6PD deficiency includes?

A

Heinz Bodies
Bite cells

33
Q

Recommended screening test for G6PD deficiency?

A

G6PD fluorescent spot test

34
Q

A screening test for G6PD deficiency

A

Autohemolysis test

35
Q

Pattern of autohemolysis associated with G6PD deficiency?

A

Autohemolysis is slightly to moderately increased but is partially corrected by glucose. [TYPE I]

36
Q

Confirmatory test for G6PD deficiency?

A

Quantitative G6PD assay

37
Q

*Classification of G6PD Deficiency Variants by the WHO

A

Class I
Class II
Class III
Class IV
Class V

38
Q

G6PD Enzyme Activity of CLASS I

A

SEVERELY deficient:

<1% activity or not detectable

39
Q

Clinical Manifestations of CLASS I

A
  • Chronic
  • Rare
  • HNSHA
  • Severity is variable
40
Q

Examples of Variants of CLASS I

A

G6PD-Serres

G6PD-Madrid

41
Q

G6PD Enzyme Activity of CLASS II

A

SEVERELY deficient:

<10% activity

42
Q

⭐️ Clinical Manifestations of CLASS II

A
  • FAVA BEANS
  • SOYA
  • MENTHOL
  • Severe
  • Episodic acute hemolytic anemia associated with infections and Certain drugs
  • NOT self-limited and may require transfusions during hemolytic episodes
43
Q

Examples of Variants of CLASS II

A

G6PD-Mediterranean

G6PD-Chatham

44
Q

⭐️ Only a small group of G6PD-deficient individuals demonstrate this,and most of these have the G6PD-Mediterranean variant?

A

FAVISM

(Unusual sensitivity to FAVA BEANS)

45
Q

G6PD Enzyme Activity of CLASS III

A

MILD to MODERATE deficient:

10% to 60% activity

46
Q

Clinical Manifestations of CLASS III

A
  • Episodic
  • Acute hemolytic anemia associated with infections and certain drugs
  • Self-limited
47
Q

Examples of Variants of CLASS III

A

G6PD- A-

G6PD-Canton

48
Q

G6PD Enzyme Activity of CLASS IV

A

MILDLY deficient to NORMAL:

60% to 150% activity

49
Q

Clinical Manifestations of CLASS IV

A

NONE

50
Q

Examples of Variants of CLASS IV

A

G6PD-B (wildtype)

G6PD- A+ (may also manifest as CLASS III)

51
Q

G6PD Enzyme Activity of CLASS V

A

INCREASED:

> 150% activity

52
Q

Clinical Manifestations of CLASS V

A

NONE

53
Q

Examples of Variants of CLASS V

A

Not reported

54
Q

removal of a part of the RBC

A

PITTING

55
Q

removal of the whole RBC

A

CULLING

56
Q

⭐️ MUST BE AVOIDED in G6PD Deficiency Patients:

A
  • SOYA
  • MENTHOL
  • NAPHTHALENE (mothballs) ⭐️
  • Dapsone
  • Methylthioninium chloride (methylene blue)
  • Nitrofurantoin
  • Phenazopyridine
  • Primaquine
  • Rasburicase
  • Tolonium chloride (toluidine blue)
  • Aniline dyes
  • Fava beans
  • Red wine
  • Legumes (ex.: garbanzos, kadyos, munggo)
  • Blueberry
  • Ampalaya
  • Cotrimoxazole
  • Quinolones
  • Sulfadiazine
  • Some herbal supplements
57
Q

Maintains iron in the HEME (Hb) in its reduced state (ferrous - Fe^2+)

A

Methemoglobin Reductase Pathway

58
Q

Methemoglobin reductase AKA?

A

Cytochrome b5 reductase

59
Q

⭐️ For the production of 2,3 - BPG

A

Rapoport-Luebering Shunt

60
Q

In Rapoport-Luebering Shunt, 2,3 - BPG binds to ____________ and DECREASES the oxygen affinity of Hb.

A

Hemoglobin

(If attached to Hgb the oxygen will be released)

61
Q

Two variables affecting the degree of association or dissociation between oxygen and hemoglobin:

A
  • partial pressure of oxygen
  • affinity of hemoglobin for oxygen
62
Q

The AFFINITY of hemoglobin for oxygen is dependent on 5 factors:

A
  1. pH
  2. Partial pressure of carbon dioxide
  3. Concentration of 2,3-bisphosphoglycerate (2,3-BPG)
  4. Temperature
  5. Presence of other hemoglobin species that are nonfunctional
63
Q

What is the curve produced when the 2 variables (partial pressure of oxygen and affinity of hemoglobin for oxygen) are PLOTTED ON A GRAPH (oxygen saturation of hemoglobin versus the partial pressure of oxygen)?

A

OXYGEN DISSOCIATION CURVE

64
Q

A shift in the curve due to an alteration in pH (or hydrogen ion concentration); and the effect of hydrogen ions and CO2 on the affinity of hemoglobin for oxygen?

A

Bohr effect

65
Q

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

A

Haldane effect

66
Q

SHIFT to the LEFT

A

↑pH

↓PCO2

↓2,3 - BPG

↓Temperature

INCREASED (↑) AFFINITY [how attached the O2 is to the Hgb]

67
Q

SHIFT to the RIGHT

A

↓pH

↑PCO2

↑2,3 - BPG

↑Temperature

DECREASED (↓) AFFINITY

Hema 1 (2 decks)

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