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Hema 1 > ERYTHROCYTES (Part 5) > Flashcards

ERYTHROCYTES (Part 5) Flashcards

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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
What is the meaning of NADPH?
Nicotinamide adenine dinucleotide phosphate
26
Can also protect the HEME IRON but less effective than the MRP/MRS?
HMS
27
Prevents the denaturation of the GLOBIN by oxidation?
HMS
28
Functionally dependent on G6PD (important to HMS to operate normally)?
HMS
29
NADPH reduces to?
GLUTATHIONE (reduced glutathione reduces peroxides and guards proteins, lipids, and heme iron from oxidation)
30
Most common enzyme deficiency in the pentose phosphate pathway?
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
31
MOST COMMON RBC enzyme defect (prevalence of 5% of the global population, or approximately 400 million people worldwide)?
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
32
Possible PBS findings of G6PD deficiency includes?
Heinz Bodies Bite cells
33
Recommended screening test for G6PD deficiency?
G6PD fluorescent spot test
34
A screening test for G6PD deficiency
Autohemolysis test
35
Pattern of autohemolysis associated with G6PD deficiency?
Autohemolysis is slightly to moderately increased but is partially corrected by glucose. [TYPE I]
36
Confirmatory test for G6PD deficiency?
Quantitative G6PD assay
37
*Classification of G6PD Deficiency Variants by the WHO
Class I Class II Class III Class IV Class V
38
G6PD Enzyme Activity of CLASS I
SEVERELY deficient: <1% activity or not detectable
39
Clinical Manifestations of CLASS I
- Chronic - Rare - HNSHA - Severity is variable
40
Examples of Variants of CLASS I
G6PD-Serres G6PD-Madrid
41
G6PD Enzyme Activity of CLASS II
SEVERELY deficient: <10% activity
42
⭐️ Clinical Manifestations of CLASS II
- 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
Examples of Variants of CLASS II
G6PD-Mediterranean G6PD-Chatham
44
⭐️ Only a small group of G6PD-deficient individuals demonstrate this,and most of these have the G6PD-Mediterranean variant?
FAVISM (Unusual sensitivity to FAVA BEANS)
45
G6PD Enzyme Activity of CLASS III
MILD to MODERATE deficient: 10% to 60% activity
46
Clinical Manifestations of CLASS III
- Episodic - Acute hemolytic anemia associated with infections and certain drugs - Self-limited
47
Examples of Variants of CLASS III
G6PD- A- G6PD-Canton
48
G6PD Enzyme Activity of CLASS IV
MILDLY deficient to NORMAL: 60% to 150% activity
49
Clinical Manifestations of CLASS IV
NONE
50
Examples of Variants of CLASS IV
G6PD-B (wildtype) G6PD- A+ (may also manifest as CLASS III)
51
G6PD Enzyme Activity of CLASS V
INCREASED: >150% activity
52
Clinical Manifestations of CLASS V
NONE
53
Examples of Variants of CLASS V
*Not reported*
54
removal of a part of the RBC
PITTING
55
removal of the whole RBC
CULLING
56
⭐️ MUST BE AVOIDED in G6PD Deficiency Patients:
- 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
Maintains iron in the HEME (Hb) in its reduced state (ferrous - Fe^2+)
Methemoglobin Reductase Pathway
58
Methemoglobin reductase AKA?
Cytochrome b5 reductase
59
⭐️ For the production of 2,3 - BPG
Rapoport-Luebering Shunt
60
In Rapoport-Luebering Shunt, 2,3 - BPG binds to ____________ and DECREASES the oxygen affinity of Hb.
Hemoglobin (If attached to Hgb the oxygen will be released)
61
Two variables affecting the degree of association or dissociation between oxygen and hemoglobin:
- partial pressure of oxygen - affinity of hemoglobin for oxygen
62
The AFFINITY of hemoglobin for oxygen is dependent on 5 factors:
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
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)?
OXYGEN DISSOCIATION CURVE
64
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?
Bohr effect
65
Depicts the occurrence by which the binding of O2 to the hemoglobin promotes the release of CO2?
Haldane effect
66
SHIFT to the LEFT
↑pH ↓PCO2 ↓2,3 - BPG ↓Temperature INCREASED (↑) AFFINITY [how attached the O2 is to the Hgb]
67
SHIFT to the RIGHT
↓pH ↑PCO2 ↑2,3 - BPG ↑Temperature DECREASED (↓) AFFINITY

Hema 1 (2 decks)

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