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5. Erythropoiesis Flashcards

1
Q

The term for RBC formation

A

Erythropoiesis

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2
Q

Total mass of RBCs circulating in the peripheral blood and the bone marrow RBC precursors.

A

Erythron

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3
Q

Erythrocytes in the circulation

A

RBC mass

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4
Q

A term that describes the dynamics of RBC creation and destruction

A

Erythrokinetics

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5
Q

Production of defective erythroid precursors

A

Ineffective erythropoiesis

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6
Q

2 examples of macrocytic, normochromic anemias caused by ineffective erythropoiesis

A

folate deficiency
vitamin b12 deficiency

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7
Q

Ineffective erythropoiesis causes defective RBC precursor cells to undergo _____ in the bone marrow before they even mature to the reticulocyte cells.

A

apoptosis

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8
Q

2 examples of microcytic, hypochromic anemias due to ineffective erythropoiesis

A

thalassemia
sideroblastic anemia

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9
Q

Decrease in the number of RBC precursors in the bone marrow resulting in decreased RBC production

A

insufficient erythropoiesis

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10
Q

what type of anemia is iron deficiency

A

microcytic, hypochromic

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11
Q

type of anemia of acute leukemia and renal disease

A

normocytic, normochromic

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12
Q

examples of conditions that resulted from insufficient erythropoiesis

A

iron deficiency anemia
acute leukemia
renal disease

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13
Q

immature hematopoietic stem cells that is committed to a cell line but cannot be identified morphologically

A

progenitor cells

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14
Q

immature HSCs that are morphologically identifiable or belonging to a given cell line

A
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15
Q

immature HSCs that are morphologically identifiable or belonging to a given cell line

A

precursor cells

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16
Q

a transferrin receptor and also the earliest marker of erythroid differentiation

A

CD71

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17
Q

progenitor vs precursor

A

progenitor: morphologically unidentifiable
precursor: morphologically identifiable

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18
Q

How to measure RBC Survival

A
  1. Collect px blood sample
  2. Label RBCs with Chromium-51
  3. Inactivate excess Chromium-51 in plasma
  4. Reinject labeled RBCs to px
  5. Measure radioactivity of RBCs at intervals until 50% of activity has disappeared
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19
Q

The results for RBC survival are commonly expressed as

A

Chromium-51 half-survival time

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20
Q

Normal range for RBC survival range

A

28-38 days

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21
Q

Chief stimulatory cytokine for RBCs

A

Erythropoietin

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22
Q

EPO is the major hormone that stimulates the production of _____

A

RBCs

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23
Q

T/F
EPO is a thermolabile, dialyzable, glycoprotein hormone

A

False. Thermostable and non-dialyzable

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24
Q

Primary cell source of EPO

A

peritubular interstitial cells of the kidneys

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25
Q

primary target cells of EPO

A

BFU-E
CFU-E

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26
Q

Normally, EPO is released into the blood in response to

A

hypoxia

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27
Q

3 major effects of EPO

A
  1. early release of reticulocytes from BM
  2. reduce time needed for RBCs to mature in BM
  3. prevent apoptotic cell death
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28
Q

apoptosis rescue by EPO is the major way of increasing

A

RBC mass

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29
Q

Blood doping can lead to

A

deadly arterial and venous thrombosis

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30
Q

Illegally done by some athletes; done by using EPO injections to increase the oxygen-carrying capacity of their body

A

Blood doping

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31
Q

Blood doping is illegally done by some athletes to increase

A

stamina and endurance

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32
Q

Growth hormone and prolactin are released by

A

pituitary gland

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33
Q

Growth hormone and prolactin stimulates erythropoiesis _______

A

directly

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34
Q

Testosterone is released by the

A

testes

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35
Q

Estrogen is released by the

A

ovaries

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36
Q

estrogen inhibits erythropoiesis ______

A

indirectly

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37
Q

testosterone stimulates erythropoiesis _______

A

indirectly

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38
Q

This is the reason why men have higher threshold values for Hgb, Hct, and RBC count.

A

Men have testosterone. Testosterone stimulates erythropoiesis.
Women have estrogen. Estrogen inhibits erythropoiesis.

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39
Q

T/F
Menstruation is the main reason why women have lower threshold values for Hct, Hgb, and RBC count.

A

False

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40
Q

committed erythroid progenitor cells

A

BFU-E
CFU-E

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41
Q

BFU-E means

A

burst forming unit-erythroid

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42
Q

CFU-E means

A

colony forming unit-erythroid

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43
Q

How long does it take for the BFU-E to mature to an erythrocyte

A

18-21 days

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44
Q

____ are spent as recognizable precursor in the BM

A

6 days

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45
Q

Rubri nomenclature

A

Rubriblast-Prorubricyte-Rubricyte-Metarubricyte-Reticulocyte-Mature RBC

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46
Q

Normoblast nomenclature

A

Pronormoblast
Basophilic normoblast
Polychromatophilic normoblast
Orthochromatic normoblast
Reticulocyte
Mature RBC

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47
Q

Erythroblast nomenclature

A

Proerythroblast
Basophilic erythroblast
Polychromatophilic erythroblast
Orthochromatic erythroblast
Reticulocyte
Mature RBC

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48
Q

When reticulocytes are found in a wright-stained smear, they are called

A

polychromatophilic erythrocytes
diffusely basophilic erythrocytes

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49
Q

When diffusely basophilic erythrocytes are found using a supravital stain, they are called

A

reticulocytes

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50
Q

Rubriblast is also called

A

Pronormoblast
Proerythroblast

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51
Q

Rubriblast size

A

12-20 um

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52
Q

Number of nucleoli of rubriblast

A

1-2

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53
Q

rubriblast n:c ratio

A

8:1

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54
Q

a morphologic feature used to identify and stage RBC and WBC precursors; also a visual estimate of what area of the cell is occupied by the nucleus compared with the cytoplasm

A

n:c ratio

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55
Q

Rubriblast gives rise to

A

2 prorubricytes

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56
Q

The earliest recognizable RBC precursor using light microscope

A

Rubriblast/Pronormoblast/Proerythroblast

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57
Q

If the areas occupied by the nucleus and the cytoplasm are approximately equal, the N:C ratio is

A

1:1

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58
Q

If the nucleus takes up <50% of the area of the cell, the ratio is

A

lower

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59
Q

If the nucleus takes up >50% of the area of the cell, the ratio is

A

higher

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60
Q

refers to the blue part of the cell and is due to the acidic components

A

basophilic

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61
Q

refers to the pink part of the cell and is due to the basic components

A

Acidophilic

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62
Q

the degree of cytoplasmic basophilia correlates with the quantity of

A

ribosomal RNA

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63
Q

As the RBC matures, the eosinophilia of the cytoplasm correlates with the accumulation of

A

hemoglobin

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64
Q

Prorubricyte other names

A

Basophilic Normoblast
Early Normoblast
Basophilic Erythroblast
Early Erythroblast

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65
Q

size of prorubricyte

A

10-15 um

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66
Q

of nucleoli of early normoblast

A

0-1

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67
Q

N:C ratio of Basophilic Erythroblast

A

6:1

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68
Q

Most helpful criteria in distinguishing early normoblast from proerythroblast

A

coarser chromatin
absent nucleoli

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69
Q

Basophilic normoblast is the last stage with a

70
Q

Prorubricyte is the first stage of

A

hemoglobinization

71
Q

Rubricyte other names

A

Polychromatophilic normoblast
Intermediate normoblast
Polychromatophilic erythroblast
Intermediate Erythroblast

72
Q

Intermediate normoblast size

73
Q

of nucleolus of intermediate erythroblast

74
Q

N:C ratio of Polychromatophilic normoblast

75
Q

The prorubricyte gives rise to

A

4 rubricytes

76
Q

Each rubricyte gives rise to

A

2 metarubricytes

77
Q

What RBC precursor is commonly confused with lymphocyte

A

Polychromatic normoblast

78
Q

lymphocyte vs rubricyte

A

Lym nucleus: crushed velvet; cytoplasm: sky blue, “robin’s egg” blue
Rubricyte nucleus: checkerboard; cytoplasm: muddy/gray

79
Q

This RBC precursor is the last stage to undergo mitosis

A

Intermediate erythroblast

80
Q

The first stage where the pink color caused by recent hemoglobinization first manifests

81
Q

Metarubricyte other names

A

Orthochromatic normoblast
Late normoblast
Acidophilic normoblast
Orthochromatic erythroblast
Late erythroblast
Pyknotic erythroblast
Nucleated red blood cells

82
Q

The nucleus is _____ during the pyknotic erythroblast phase

83
Q

Last RBC precursor to have a nucleus

A

Acidophilic normoblast

84
Q

size of late erythroblast

85
Q

of nucleolus of late normoblast

86
Q

color of orthochromatic erythroblast’s cytoplasm

A

salmon-pink

87
Q

N:C ratio of Orthochromatic normoblast

88
Q

Enveloped extruded nucleus engulfed by bone marrow macrophages

A

pyrenocyte

89
Q

Frequently, small fragments of the nucleus are left behind if the nRBC projection is pinched off before the entire nucleus is enveloped. These small fragments are called

A

Howell-Jolly bodies

90
Q

Howell-Jolly bodies are removed from the RBCs by the spleen thru

91
Q

Young RBCs containing residual RNA

A

Reticulocyte

92
Q

size of reticulocyte

93
Q

What RBC precursor has the same size?

A

Metarubricyte
Reticulocyte

94
Q

of nucleolus of reticulocyte

95
Q

T/F
Reticulocyte has a nucleus

96
Q

Last immature erythrocyte stage

A

Reticulocyte

97
Q

Reticulocytes spend _____ in the bone marrow and ____ in the peripheral blood before developing into a mature RBC

A

2-3 days
1 day

98
Q

2 types of reticulocytes

A

shift cells
stress reticulocytes

99
Q

These reticulocytes are seen in cases with increased RBC production.

A

Shift cells/polychromatophilic macrocyte

100
Q

These reticulocytes are seen in severe conditions like hemolytic anemia

A

Stress reticulocytes/ Macroreticulocytes

101
Q

The last stage of hemoglobin synthesis

A

Reticulocytes

102
Q

Reticulocyte count is used to diagnose the type of _____ present in a patient

103
Q

size of mature rbc

104
Q

shape of mature rbc

A

biconcave disk

105
Q

thickness of rbc

A

1.5-2.5 um

106
Q

Average life span of rbc

107
Q

number of rbcs produced from 1 rubriblast

108
Q

Normal ratio of RBCs to WBCs

109
Q

Normal ratio of RBCs to platelets

110
Q

Adult RBC contains no mitochondria, therefore there is no

A

protein, hemoglobin synthesis

111
Q

Central pallor of mature RBC occupies ____ of the cell’s diameter1

112
Q

Major macromolecule that constitutes the RBC membrane

A

Proteins (peripheral and integral) 52%

113
Q

Other RBC constituents aside from proteins

A

lipids 40%
CHO 8%

114
Q

Provides the vertical support connecting the bilayer to the underlying cytoskeleton to maintain RBC membrane integrity

A

transmembrane proteins

115
Q

An integral protein that transports water

A

Auaporin 1

116
Q

An integral protein that transports anion and supports ABH antigens

117
Q

Ca2+-ATPase transports

118
Q

Supports Fy antigen

119
Q

Glucose transporter and supports ABH antigen

120
Q

Transports negatively charged sialic acid (3)

A

Glycophorin A
Glycophorin B
Glycophorin C

121
Q

Different functions of 3 integral proteins that transports negatively charged sialic acid

A

GA: supports MN determinants
GB: supports Ss determinants
GC: supports Gerbich system determinants

122
Q

a transmembrane protein that is responsible for integrin adhesion

123
Q

Zn2+-binding peptidase, K antigens

124
Q

Integral protein Jk transports

125
Q

D and CcEe antigens

126
Q

D and CcEe antigens

127
Q

This is necessary for expression of D and CcEe antigens, also transports gas i.e. CO2

128
Q

Proteins that provide the horizontal support for the membrane.

A

Skeletal/Cytoskeletal/Peripheral Proteins

129
Q

The shape and flexibility of the RBC depends on the

A

cytoskeleton

130
Q

primary skeletal proteins

A

a-spectrin
b-spectrin

131
Q

caps actin filament

A

adducin
tropomodulin

132
Q

Ankyrin anchors (2)

A

band 3 and protein 4.2

133
Q

actin building protein

134
Q

F-actin binds

A

b-spectrin

135
Q

anchors 4.1 complex

A

protein 4.1

136
Q

protein 4.2 anchors

A

ankyrin complex

137
Q

regulates actin polymerization

A

tropomyosin

138
Q

Defect in protein(s) that disturbs vertical membrane interactions between transmembrane proteins and underlying cytoskeleton; loss of membrane and decreased surface area-to-volume ratio.

A

Heredirary Spherocytosis

139
Q

Inheritance pattern of hereditary spherocytosis

A

autosomal dominant

140
Q

deficient proteins in hereditary spherocytosis (5)

A

ankyrin
band 3
a-spectrin
b-spectrin
protein 4.2

141
Q

this rbc index is elevated in hereditary spherocytosis

A

Mean Corpuscular Hgb Concentration

142
Q

Normal MCHC range

A

31-37 g/dL

143
Q

MCHC of px with HS

A

35-38 g/dL

144
Q

What is the only disease that is characterized by high MCHC?

A

Hereditary Spherocytosis

145
Q

T/F

In HS px, the rbc membrane is elastic, but is less stretchable

A

FALSE.

stretchable, less elastic

146
Q

Spherocytes gather this electrolyte( ?) at a higher rate than normal RBCs in the spleen; leading to a decreased tolerance to osmotic changes because they —- then lyse.

147
Q

What surgical procedure is beneficial to px with HS

A

splenectomy

148
Q

Most sensitive and specific test to confirm the diagnosis of HS

A

Eosin-5′-maleimide (EMA) binding test

149
Q

principle of EMA binding test

A

flow cytometry

150
Q

Defect in proteins that destroy the horizontal linkages in the protein cytoskeleton which results to the loss of mechanical stability of membrane

A

Hereditary elliptocytosis

151
Q

Inheritance pattern of hereditary spherocytosis

A

Autosomal dominant

152
Q

deficient proteins in HE (3)

A

a-spectrin
b-spectrin
Protein 4.1

153
Q

A rare subtype of hereditary elliptocytosis that has an autosomal recessive pattern

A

Hereditary pyropoikilocytosis

154
Q

deficient proteins in hereditary pyropoikilocytosis

A

a-spectrin
b-spectrin

155
Q

Severe defect in spectrin the disrupts horizontal linkages in protein cytoskeleton; severe RBC fragmentation

A

Hereditary pyropoikilocytosis

156
Q

Hereditary ovalocytosis is also known as

A

Southeast Asian ovalocytosis

157
Q

inheritance pattern of hereditary ovalocytosis

A

autosomal dominant

158
Q

defective protein in southeast asian ovalocytosis

159
Q

Defect in band 3 causing increased membrane rigidity; resistant to malaria; prevalent in some areas of SEA

A

hereditary ovalocytosis

160
Q

Overhydrated hereditary stomatocytosis causes increased membrane permeability to

161
Q

hereditary pattern of overhydrated hereditary stomatocytosis

A

autosomal dominant

162
Q

deficient proteins in overhydrated hereditary stomatocytosis

A

Rh-associated protein (RHAG)
others unknown

163
Q

In OHS increased intracellular Na causes influx of H2O, increase in cell volume, and decreased cytoplasmic viscosity. This further results to what RBC morphology

A

stomatocytes (5-50%)
macrocytes

164
Q

The most common form of stomatocytosis

A

Dehydrated hereditary stomatocytosis

165
Q

Dehydrated hereditary stomatocytosis is also known as

A

Hereditary Xerocytosis

166
Q

Inheritance pattern of hereditary xerocytosis

A

autosomal dominant

167
Q

deficient protein in hereditary xerocytosis

A

piezo-type mechanosensitive ion channel component 1

168
Q

In HX, there is increased permeability to (ELEC?) leading to a decrease in this intracellular electrolyte

169
Q

A decrease in intracellular K results to

A

loss of water from cell
decrease in cell volume
increased cytoplasmic viscosity

170
Q

Typical RBC morphology in HX

A

Codocytes
Burr cells
Stomatocytes (<10%)
RBCs with puddled Hgb at periphery
dessicated cells with spicules

171
Q

HS, HE, HP, HO/SEAHO are caused by mutations that change

A

membrane structure

172
Q

OHS, DHS/HX are caused by mutations that change

A

membrane transport proteins

Hematology (7 decks)

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