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Hematology 1 > (P) Week 3: RBC function Part 1 > Flashcards

(P) Week 3: RBC function Part 1 Flashcards

1
Q

Enumerate the general characteristics of normal RBC

A
  1. Bi concave disc
  2. with central pallor
  3. Average volume of 90 fL(fentoliter)
  4. 6-8 micrometer in diameter
  5. 1.5-2.5 micrometer in thickness
  6. No cytoplasmic inclusions
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2
Q

abnormal shaped RBC

A

Poikilocytosis

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3
Q

depression in the center of the RBC where the nucleus was located

A

central pallor

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4
Q

normal percentage of the central pallor size

A

30-45% of the total diameter of the rbc

other souces say not more than 3um or 1/3 of the total diameter

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5
Q

RBC where the central pallor is smaller or absent

found in megaloblastic anemia

A

hyperchromic red cells

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6
Q

RBC’s central pallor is larger than the reference value

found in iron-deficiency anemia

A

hypochromic red cells

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7
Q

what is the ave diameter of a RBC

A

7.2-7.5 micrometers (range is 6-8 micrometers)

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8
Q

RBC that has a diameter of below 6 micrometers

A

microcytic RBC

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9
Q

RBC that has a diameter of more than 8 micrometers

A

macrocytic rbcs

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10
Q

RBC have varying size in general

A

anisocytosis

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11
Q

what is the average surface area of a rbc

A

140 micrometers

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12
Q

the ability of a RBC to be flexible

A

deformability

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13
Q

components of a RBC

A

membrane structure
Hgb stability
energy production

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14
Q

hemolysis that occurs outside the blood vessels, usually within the reticuloendothelial system such as the spleen and liver

A

extravascular hemolysis

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15
Q

red cell destruction in the blood vessels

A

intravascular hemolysis

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16
Q

What are the components of a RBC membrane

A

proteins
carbohydrates
lipids

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17
Q

what are the two types of proteins that make up the protein part of the RBC membrane

A

transmembrane (integral proteins) and the peripheral proteins

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18
Q

this is the point of attachment, vital to the stability of the RBC membrane structure

A

peripheral proteins

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19
Q

proteins that passes through the bi-lipid layer

A

transmembrane

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20
Q

lipids that make up the rbc membrane includes

A

cholesterol and phospolipid

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21
Q

the lipid bi-layer cosists of ______ heads and a ______ tail.

A

polar head
non-polar tail

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22
Q

The polar heads of the
phospholipid bi-layer is______
non-polar tail is ______

A

hydrophobic and hydrophilic

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23
Q

provides additional cushion in the red cell membrane, is situated within the lipid layer

A

cholesterol

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24
Q

disorder where cholesterol membrane is affected due to lack of beta-lipoprotein

A

abetalipoproteinemia

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25
Q

vital for the continuous metabolism of cholesterol

A

beta-lipoprotein

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26
Q

a kind of poikilocytosis associated with abetalipoproteinemia as there is a deviation in the shape of the RBC

A

acanthocytosis

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27
Q

Transmembrane proteins carry the blood group antigens, they are the one that dictates our blood types

A

Blood group Ag

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28
Q

what do you lack if you do not have a GPI anchor

A

GPI-linked protein

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29
Q

defective __________ is associated with an oblong / oval ellipsoidal RBC

A

Spectrin-Ankyrin-Protein-Band 4.1

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30
Q

Issues with Spectrin-Ankyrin-Protein-Band 4.1 leads to this disease characterized with oval shaped RBCs

A

elliptocytosis

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31
Q

3 diseases associated with defective Spectrin-Ankyrin-Protein-Band 4.1

A

Hereditary elliptocytosis, pyropoikilocytosis, and leach phenotype

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32
Q

defective ____________ causes the RBC to become loose and form abnormal blebs or mud-like structures.

A

Spectrin-Ankyrin-Protein-Band 3-4.2

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33
Q

what kind of abnormal shape is observed if the problem is Spectrin Ankyrin-PB3-PB4.2.

A

spherocytes or hereditary spherocytosis

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34
Q

Defective _________ may be associated with hereditary ovalocytosis / southeast asia ovalocytosis

A

Spectrin Ankyrin-PB3

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35
Q

Spectrin protein is made up of which two chains?

A

alpha and beta chain

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36
Q

responsible for the formation of Glycosylphosphatidylinositol linked proteins (GPI-linked proteins) in our RBC

A

PIGA gene

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37
Q

Paroxysmal nocturnal hemoglubinuria (PNH) is due to the deficiency of the of the following:

A

PIGA gene
CD 55
CD 59

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38
Q

also known as the Decaying Accelerating Factor

A

CD 55

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39
Q

also known as the Membrane Inhibitor Reactive Lysis Protein

A

CD 59

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40
Q

What is that condition that is associated with
problems with your GPI protein?

A

Paroxysmal nocturnal hemoglobinuria (PNH)

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41
Q

means that sometimes there are visible symptoms in the patient or sometimes there are none

A

paroxysmal

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42
Q

a condition where the red cells are destroyed and hemoglobin are liberated in the blood and is reflected on the urine

A

hemoglobinuria

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43
Q

other name for protectin

A

MAC inhibitor protein

44
Q

prevents the complete formation of the
“membrane attack complex”, (known also as
C5b6789)

A

MAC inhibitor protein or protectin

45
Q

how does the MAC inhibitor protein or protectin prevent the formation of the membrane attack complex

A

preventing the attachment of c9 protein

46
Q

where are complement proteins normally found?

A

plasma or blood

47
Q

_________ fights bacteria alongside WBCs by coating the it and punching a hole on the bacterial membrane

A

complement proteins

48
Q

How does the RBCs protect themselves from being collateral damage from complementary proteins attacking bacteria?

A

CD 55 and 59 protects the RBC

49
Q

What state/ form does our hemoglobin take form in to prevent being precipitated?

A

fluid / reduced state

50
Q

how many globin chains is in a single hemoglobin

A

2 pairs

51
Q

how many heme is found in a single hemoglobin

A

four

52
Q

a vital part of the hemoglobin molecule that helps in the normal and efficient release of oxygen from the hemoglobin of the tissue

A

2,3 DPG

53
Q

where is the 2,3 DPG synthesized

A

embden meyrehof metabolic pathway (Rapaport-Leubering pathway to be more specific)

54
Q

An inclusion formed in the hemoglobin due to oxidization

A

Heinz bodies

55
Q

In what state does heme have to be so that our body may absorb its iron content?

A

Ferrous state (Fe^2+)

56
Q

What are the two components of heme?

A

protoporphyrin IX
Fe^2+

57
Q

what enzyme aids the insertion of Fe^2+ into the protoporphyrin IX

A

ferrochelatase

58
Q

T or F

Iron is absorbed in the ferric state (Fe^3+) from our diet

A

F (ingested only in the ferric state but it is then converted to the ferrous state to be absorbed)

59
Q

State in which iron is stored

A

ferritin

60
Q

ferritin is composed of this protein along with a ferric iron

A

apoferritin

61
Q

apoferritin is a special kind of protein present on some human cells that received iron from the blood-ferritin transport protein known as __________

A

Transferrin

62
Q

alpha and zeta genes are found in

A

chromosome 16

63
Q

Beta, delta, gamma, and epsilon genes are carried by

A

chromosome 11

64
Q

We can measure how much is our A1, A2, and F through

A

electrophoresis

65
Q

the molecular structure of the globin chains forms a _________ appearance which forms loops and folds within it called helices

A

quarternary appearance

66
Q

When you see this card please study heme formation

familiarize the stps:

A
  1. glycine + succinyl CoA
  2. Goes outside the cytoplasm, and is changed into porphobilinogen by d-Amino-levulinic acid
  3. transformed to hydroxymethyl bilane by PB deaminase
  4. changed into Uroporphyrinogen III by Uro II synthase
  5. changed to coproporphyrinogen III by Uro III decarboxylase (enters the mitochondria again)
  6. Transformed into Protoporphyrinoggen III by CP III oxidase
  7. end point is heme ring / Protoporphyrin IX by protoporpyrin III oxidase enzyme
67
Q

T or F

Heme ring is not considered as a heme because it contains iron

A

T

Heme ring = no iron
heme = iron baby

68
Q

The transport protein for iron

A

Transferrin

69
Q

What is the form of iron in the blood?

A

ferric

70
Q

the binding of the transferrin protein

A

transferrin ferric complex

71
Q

term used when transferrin passed the iron to the apoferritin

A

apoferritin ferric complex

72
Q

maturing red cells esp rubriblast will adhere to the _________ to ask for iron

A

macrophages

73
Q

which phase does the formation of hemoglobin (binding of the heme and globin) in the cytoplasm occur ?

A

rubricyte stage

74
Q

what is the last stage wherein hemoglobin is formed

A

reticulocyte

75
Q

type of porphyria where ferrochelatase is affected, it is dominant, photosensitive, an increase in protoporphyrin is observed in the blood and feces

A

erythropoetic porphyria

76
Q

type of porphyria that lacks uro III synthase, and is recessive

A

congenital erythropoietic porphyria

77
Q

type of porphyria that lacks ALA-synthase, passed down as an X-linked trait

A

X-linked protoporphyria

78
Q

T or F

porphyrins will be excreted by the body as the formation of heme is interrupted by the affected enzymes

A

F (it will accumulate)

79
Q

a heme kinetic problem that will lead to severe anemia and photophobia, aka Vampire disorder

A

porphyria

80
Q

porphyrins are seen and deposited to _____ and _____ in porphyria

A

teeth and urine

81
Q

2 metabolic pathways that the red cells utilize

A

Embden Meyerhof Pathway (EMP)
Hexose monophosphate pathway (HMP)

82
Q

Glycolitic pathway whose main responsibility is to produce energy anaerobically from antioxidants derived from drugs and toxic substances

A

EMP

83
Q

how many ATPs are used by the EMP

A

2

84
Q

How many ATPs are produced by the EMP?

A

4

85
Q

What is the net ATP of EMP?

A

2 (4 are produced BUTTT 2 are used, therefore making the net ATP 2 lang :>)

86
Q

enzymes used in the first phase of EMP glucose metabolism

A

hexokinase
gluconate phosphate isomerase
Phosphofructokinase

(Hey gurl posey)

87
Q

enzymes used in the second phase of EMP glucose metabolism

A

Aldolase
Triose Phosphate isomerase
Phosphoglycerate

(ATP)

88
Q

enzymes used in the third phase of EMP glucose metabolism

A

3-phosphoglycerate mutase
enolase
pyruvate kinase
lactate dehydrogenase

three enormous pussy lactate ehe

89
Q

what is the end point of glucose metabolism

A

lactate

90
Q

T or F

the embden myerhof pathway does not use any oxygen

A

T

91
Q

ATP will not be produced if you have what type of deficiency, therefore stopping the rbc metabolism

A

Pyruvate kinase deficiency

92
Q

around how many percent of the glucose enter the embden-meyerhof pathway for energy production

A

90-95%

93
Q

pyruvate kinase deficiency changes the rbc into what abnormal shapes?

A

burr cells / acanthocytes

94
Q

What is the main objective of the Embden-Meyerhof pathway?

A

produce energy

95
Q

The site of synthesis for 2,3-Diphosphoglycerate (DPG)

A

Rapport-leubering pathway

96
Q

This is a very important hemoglobin component, it pushed out the oxygen from our hemoglobin to the tissues

A

2,3-diphosphoglycerate (DPG)

97
Q

what is the condition secondary to 2,3-diphosphoglycerate deficiency

A

hypoxia

98
Q

A hemoglobin that carries a ferric (Fe3+) iron

A

methemoglobin

99
Q

Iron becomes ferric due to exposure to toxic substances such as ______ and _______

A

nitrates and nitrites

100
Q

What will you feel if your methemoglobin is at 1%

A

nothing, you’re still fine as methemoglobin should be around <1

101
Q

what will you feel if methemoglobin levels reaches 3%

A

cyanosis and hypoxia

102
Q

This enzyme allows the iron in its ferric state return to its ferrous state, as well as provides protection against oxidant present in the food we eat

A

methemoglobin reductase enzyme

103
Q

this pathway is responsible for reducing the ferric iron into ferrous iron within the heme

A

methemoglobin reductase pathway

104
Q

other name for methemoglobin reductase

A

cytochrome b5 reductase

105
Q

you’ve reached the 105th card if you’re a concrete sequential learner !!

A

pray for nath’s sanity as she tries to keep the cards up to date !!!

Hematology 1 (14 decks)

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