Hematology Exam 2 Flashcards

1
Q

Stain used to view blood smears and bone marrow

A

Wright’s Stain

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2
Q

Stain to view reticulocytes

A

New Methylene Blue

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3
Q

What type of stain is New Methylene Blue?

A

Supravital

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4
Q

Stain used to view iron

A

Prussian Blue

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5
Q

A normocytic, normochromic erythrocyte typically measures at how many microns?

A

7 microns

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6
Q

Central pallor of erythrocytes is typically __/__ of the cell

A

1/3

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7
Q

TRUE or FALSE

Even if only one or two cells are seen to have abnormalities (ex. echinocyte), you will make note of it on the differential.

A

FALSE

In order to be clinically significant, MOST abnormal morphology needs to be seen in every field.

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8
Q

DEFINITION:

Variation in size

A

Anisocytosis

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9
Q

DEFINITION:

Variation in shape

A

Poikilocytosis

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10
Q

DEFINITION:

Size of RBC

(indices)

A

Mean Cell Volume

(MCV)

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11
Q

DEFINITION:

Average weight of hemoglobin in red blood cell

(indices)

A

Mean Cell Hemoglobin

(MCH)

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12
Q

DEFINITION:

Average concentration of hemoglobin in each red blood cell

(indices)

A

Mean Cell Hemoglobin Concentration

(MCHC)

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13
Q

TRUE or FALSE

Spherocytosis is the only case that a smear would be called hyperchromic

A

TRUE

Otherwise it doesn’t ‘really’ exist (misnomer)

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14
Q

DEFINITION:

Pale blue-gray erythrocytes (Wright’s Stain)

A

Polychromasia

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15
Q

DEFINITION:

Pale blue-gray erythrocytes (New Methylene Blue Stain)

A

Reticulocytes

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16
Q

What is the size of most erythrocytes?

A

6-8 microns

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17
Q

What is the normal range for MCV?

A

80-100 fL

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18
Q

What do we typically compare RBCs to for reference on size?

A

The nucleus of a normal ‘resting’ lymphocyte

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19
Q

MCV>100 should typically show what type of RBCs under a microscope?

A

Macrocytes

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20
Q

What intracellular issue typically causes Macrocytes?

A

Impaired DNA synthesis in the nucleus

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21
Q

What is typically happening to cause macrocytes in the blood?

A

>1% of RBCs are being destroyed

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22
Q

Changes in membrane lipids causing the cell to spread-out (macrocytes) can be seen in which disease?

A

Liver disease

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23
Q

MCV<80fL should show what type of RBCs in a peripheral smear?

A

Microcytes

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24
Q

Intracellular cause of microcytes

A

Impaired hemoglobin synthesis

-Any deprivation of hemoglobin molecules will cause an increase in cell division, decreasing cell size.

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25
Examples of impaired hemoglobin synthesis that will cause microcytes (3)
**-Thalassemia - involves globin chain** **-Iron deficiency anemia (IDA)** **-Lead poisoning - interferes with heme synthesis**
26
RBC Shape: - Rod or oval - oval shape increases as cell matures - Hereditary or acquired condition - Defect in spectrin affecting the horizontal linkage of cell cytoskeleton
**Ovalocytes** **(Elliptocytes)**
27
You can see ovalocytes (elliptocytes) for many reasons. How many can you remember? (6 given in lecture)
**-Hereditary ovalocytosis (if majority are oval)** **-Sickle cell anemia** **-Iron deficiency anemia** **-RBC enzyme deficiences** **-Pernicious anemia as macro-ovalocytes** **-Miscellaneous anemia (non-specific)**
28
Another name for ovalocytes
**Elliptocytes**
29
Another name for sickle cells
**Drepanocytes**
30
Abnormal hemoglobin S causes this type of RBC shape
**Sickle cell**
31
This form of hemoglobin can cause the following: - Decreased solubility - Homozygous causes severe hemolytic anemia - Heterozygous have both normal and abnormal hemoglobin - Sickling occurs when oxygen tension decreases
**Hemoglobin S**
32
Drepanocytes are specific for which disease?
**Sickle Cell Disease**
33
TRUE or FALSE You WILL report drepanocytes even if only a small number of them are seen
**TRUE** **Drepanocytes = Sickle cells**
34
RBC Shape: - Small spherical cells with pointed irregular spaced projections radiating from cell tips - Tips may be bent back or are bulb-like - 3 or more blunt spicules of uneven length - Cells have no central pallor
**Spur cells** **AKA** **Acanthocytes**
35
An altered lipid content (increased cholesterol) can cause this RBC shape
**Acanthocytes** **AKA** **Spur cells**
36
When are acanthocytes most often observed in peripheral smears? (4 given in lecture)
**-Liver disease** **-Congenital Abetalipoproteinemia (rare)** **-Alcoholic liver disease** **-Post-splenectomy**
37
RBC Shape: - "Sea urchin" - 10-30 rounded spicules evenly placed over the surface of the erythrocyte - Spicules may be even, sharp, and random
**Echinocytes** **AKA** **Burr/Crenated cells**
38
When are echinocytes observed? | (5 ways given in lecture)
**-Liver disease** **-Uremia (increased blood urea due to kidney disease)** **-Dehydration** **-Peptic ulcers** **-Pyruvate Kinase Deficiency**
39
TRUE or FALSE Burr cells are caused due to a change in the tonicity of the cell when there is an anion imbalance
**FALSE** **Burr cells occur due to a cation imbalance (sodium and potassium)**
40
TRUE or FALSE It isn't necessary to call schistocytes unless you see a lot of them
**FALSE** **Always make note of schistocytes**
41
RBC Shape: - Fragments are formed as RBC is abnormally desrtoyed - RBC may become damaged as it squeezes through small damaged vessel
**Schistocytes**
42
What were the 5 reasons given in lecture for why we might see schistocytes in a peripheral smear?
**-Coagulation defects such as DIC** **-Microangiopathic hemolytic anemia** **-Heart-valve hemolysis** **-Severe burns (heat damages spectrin in membrane)** **-Uremia**
43
The helmet cell, aka keratocyte, is a variant form of which abnormal RBC shape?
**Schistocyte**
44
Another name for Dacryocyte
**Tear Drop Cell**
45
RBC Shape: - Round cell with a single elongated or pointed extremity - Pear-shaped
**Dacryocytes** **AKA** **Tear Drop Cell**
46
What would cause us to see dacryocytes? (4 ways given in lecture)
**-Myelofibrosis (fibrous material in bone marrow)** **-Extra-medullary hematopoiesis** **-Enlarged spleen or removal of inclusion from a cell by the spleen (macrophages try to grab RBC but RBC escapes)** **-Megaloblastic Anemia**
47
RBC Color: - Central pallor \>3microns - Due to impaired hemoglobin synthesis - Usually observed alongside microcytosis - Can be measured with MCH and MCHC - May be observed due to water artifact
**Hypochromasia**
48
RBC Color: - Term used to describe the blue-gray RBCs observed on Wright Stain - Normally will observe ~1% of RBCs looking this way - If increased number is observed, indicates the bone marrow is increasing output of cells
**Polychromasia**
49
Where are the highest number of polychromatic cells is observed? (4 given in lecture)
**-Post hemorrhage** **-Hemolytic anemia** **-Significant loss of RBCs** **-Following treatment of iron deficiency anemia**
50
- Increased production of RBCs with no central pallor - Defect in membrane protein causing vertical disruption between transmembrane proteins and underlying cytoskeleton - Only cell described as hyperchromic - Decreased surface area to volume ratio - Causes extravascular hemolysis - MCHC \>36% - MCV ~80fL
**Spherocytosis**
51
RBC Shape: - Cell loses part of its membrane from antibody or genetic defect - May be hereditary or due to an immune hemolytic anemia - ANY observed is considered clinically significant
**Spherocytes**
52
What is always considered clinically significant in a peripheral blood smear?
**-Parasites** **-Sickle cells** **-Schistocytes** **-Spherocytes**
53
Abnormal RBC: - Bell shaped - Appear very thin and flat on smear - "Bull's eye"
**Target Cell** **AKA** **Codocyte**
54
Another name for Target Cell
**Codocyte**
55
What causes codocytes?
**A rearrangement of hemoglobin**
56
This abnormal RBC has an increased cell membrane and surface area due to lipid alteration causing the cells to expand.
**Codocytes** **AKA** **Target Cells**
57
TRUE or FALSE Codocytes cause decreased hemoglobin leading to thalassemia and severe cases of iron deficiency anemia
**TRUE**
58
What are some disorders that can cause an increase in membrane lipids leading to viewing codocytes on a peripheral smear? (4 given in lecture)
**-Liver disease (aquired)** **Hemoglobinopathies such as:** **-Sickle cell anemia** **-Hemoglobin C disease** **-Thalassemia**
59
Abnormal RBC: - Cup shaped - "Kiss cell" - Often is an artifact - Central pallow is slit-shaped or rectangular
**Stomatocytes**
60
TRUE or FALSE An electrolye imbalance causing potassium to be increased and sodium to be decreased can produce stomatocytes in a peripheral smear
**False** **the imbalance is from increased sodium and decreased potassium**
61
TRUE or FALSE Stomatocytes are observed in alcoholism
**TRUE**
62
RBC inclusion: - Remnants of nuclear material (DNA) - Small deep blue, round granule typically in the periphery of the cell
**Howell-Jolly Body**
63
Cause of Howell-Jolly Bodies | (2 reasons given in lecture)
**-Accelerated or abnormal erythropoiesis** **-Spleen not removing (pitting) piece of nucleus** **Non-functioning spleen is the most common reason**
64
Howell-Jolly Bodies are observed in which type of Megaloblastic anemias?
**-B12 deficiency** **-Folic acid deficiency** **Faulty maturation of RBC precursors**
65
TRUE or FALSE Splenomegaly or splenectomy can cause Howell-Jolly bodies to be seen in peripheral blood smears
**TRUE** **non-functioning spleen is the most common reason we see Howell-Jolly Bodies**
66
RBC inclusion: - Aggregates or clumped ribosomes containing RNA (denatured RNA) - Indicates altered or impaired hemoglobin synthesis - Granules are distributed throughout the RBC (either fine or coarse)
**Basophilic stippling**
67
What causes basophilic stippling granules to appear fine/small?
**Thalassemia**
68
What causes basophilic stippling granules to appear coarse?
**Lead poisoning**
69
RBC inclusion: - Collection of mitochondria and ribosomes that contain iron - Contain non-heme iron (Fe3+) that was not utilizedin hemoglobin production (due to ineffective iron metabolism)
**Pappenheimer Bodies**
70
RBC inclusion: - on Wright's stain - subtle pale dots near periphery of cell - Typically appear in doublets or triplets
**Pappenheimer Bodies**
71
Confirmation stain for Pappenheimer bodies
**Prussian Blue** **or** **Iron Stain**
72
RBC inclusion: This inclusion is seen in sideroblastic anemia
**Pappenheimer bodies**
73
Type of anemia: - Iron overload - Overabundance of iron metabolism causing iron to be stored in other places besides the liver and bone marrow
**Sideroblastic anemia**
74
When would we see pappenheimer bodies? | (3 possibilities discussed in lecture)
**-Sideroblastic anemia** **-Thalassemia** **-Splenectomy**
75
When using Prussian Blue stain, pappenheimer bodies are called \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
**Siderocytes**
76
Definition: Non-nucleated red blood cell in which particles of iron are visible with Prussian Blue stain
**Siderocyte**
77
Definition: Nucleated red blood cell precursor that shows excessive iron granules with Prussian Blue stain
**Sideroblast**
78
Definition: Nucleated red blood cell precursor with at least five granules that circle at least one third of the nucleus with Prussian Blue stain
**Ringed Sideroblast**
79
RBC inclusion: - Not seen with Wright Stain - Must use Supravital stain
**Heinz Bodies**
80
Types of supravital stains (2)
**-New Methylene Blue** **-Crystal Violet**
81
RBC inclusion: - Composed of denatured or precipitated hemoglobin - Large round masses lying under or attached to the cell membrane
**Heinz Bodies**
82
RBC inclusion: Observed in: - G6PD deficiency - Unstable hemoglobin, causing iron to be unprotected - Oxidizing drugs - Alpha thalassemia
**Heinz Bodies**
83
RBC inclusion: - Ring or figure 8 forms - Contain arginine-rich histone and non-hemoglobin iron - Thought to be remnants of spindle fibers, which form during mitosis - _Very rare_
**Cabot Rings**
84
RBC inclusion: Observed in: - Severe anemias - Pernicious anemia - Dyserythropoiesis
**Cabot Rings**
85
RBC inclusion: - Oblong dark crystal - Not normally present in normal adult blood - Diagnostic - Abnormal hemoglobin crystallizes
**Hemoglobin C Crystals**
86
RBC inclusion: - Indicaties infectin with one of four species of parasite - Stages observed is dependent on type with ring forms being most common - Must be careful to not confuse with platelet on top of RBC - _Always clinically significant even if only 1 is seen_
**Malaria**
87
4 different species of Malaria
**-P. malariae** **-P. falciparum** **-P. vivax** **-P. ovale** **(P = Plasmodium)**
88
RBC inclusion: - Always abnormal in adult peripheral blood - Few are normally observed in newborn blood (2-24/100 WBC on newborn) - Typically a metarubricyte
**Nucleated Red Blood Cell** **-NRBC-**
89
2 situations that would cause RBCs to clump
**-Agglutination** **-Rouleaux**
90
Red Cell Distribution: - Random clumping of RBCs - Usually due to a cold antibody - MCV increases, RBC count decreases, MCHC \>38%
**-Agglutination**
91
Red Cell Distribution: These antibodies will stop agglutinating at 37C (Body temp)
**IgG**
92
Red Cell Distribution: These antibodies will stop agglutinating at 25C (Room temp)
**IgM**
93
Red Cell Distribution: What is suggested for agglutination?
**Warm specimen to 37C**
94
Red Cell Distribution: - Red blood cells look like stacks of coins - Due to elevated antibodies (globulins) or improper balance of proteins in serum - Thick parts of normal peripheral smears can look this way
**Rouleaux**
95
Red Cell Distribution: An improper balance of these globulins/proteins would likely cause rouleaux on a peripheral smear (3)
**-IgG** **-IgA** **-Fibrinogen**
96
Red Cell Distribution: When could you encounter rouleaux? (3)
**-Multiple myeloma** **-Liver disease** **-Chronic inflammation**
97
Types of RBCs that are diagnostic even if only a few are seen (7)
**-Schistocytes** **-Sickle cells** **-C crystals** **-Malaria/other blood parasites** **-Target cells if anemia is present** **-Tear drops cells in some settings** **-Spherocytes in some settings (severe burn)**
98
- Simple, non-specific screen for inflammation in hematology - Anticoagulated blood stands undisturbed for 1 hour - Distance in millimeter that RBCs will fall in one hour in a vertical tube of defined length
**ESR** **Erythrocyte Sedimentation Rate**
99
Determines presence of anemia
**Hemoglobin** **and** **Hematocrit**
100
Cloud of negative charges found on RBC cell membrane
**Zeta**
101
- Increased fibrinogen - In vivo rouleaux formation of cells - Anemia - Macrocytosis - Hypercholesterolemia - Hyperfibrinogenemia - Hypergammaglobulinemia - Leukemia - Diabetes Mellitus How would all of the above affect an ESR?
**Increase fall-out** **(Settle faster)**
102
- Polycythemia - Sickle cells - Spherocytes - Leukocytosis How would all of the above affect an ESR?
**-Decrease Fall-Out** | (settle slower)
103
Normal ESR values for Females and children
**0-20 mm/hr**
104
Normal ESR values for males
**0-15 mm/hr**
105
2 commonly used methods for ESR
**Wintrobe** **Westergren**
106
ESR method: - Utilizes a 100mm column - Allows for increased sensitivity in detecting mildly elevated ESRs
**Wintrobe method**
107
ESR method: - Considered universal reference method - Uses 200mm column with 2.55mm internal diameter - Allows for the detection of highly elevated ESR
**Westergren method**
108
Increase or Decrease ESR: Vertical slant
**Falsely increase**
109
Increase or Decrease ESR: Temperature higher that room temp (25C)
**Falsely increase**
110
Increase or Decrease ESR: Temperature lower than room temperature (25C)
**Falsely decrease**
111
Increase or Decrease ESR: Bubbles
**Falsely decrease**
112
Increase or Decrease ESR: Abnormal shapes such as sickle cell or spherocytes
**Falsely decrease** **(won't rouleaux)**
113
Increase or Decrease ESR: Improper anticoagulants (Sodium oxalate, potassium oxalate, and heparin)
**Falsely increase** **(causes RBCs to shrink)**
114
Increase or Decrease ESR: Concentration of anticoagulant -tube not properly filled
**Falsely increases** **(causes cells to alter shape - spherocytes)**
115
ESRs may be increased which cancers? | (2)
**-Multiple myeloma** **-Leukemia**
116
Increase or Decrease ESR: Hypercholesterolemia
**Increased**
117
Increase or Decrease ESR: Viral or bacterial infection
**Increased**
118
Increase or Decrease ESR: Rheumatoid arthritis
**Increased during flare up**
119
Increase or Decrease ESR: Osteoarthritis
**Increased**
120
Increase or Decrease ESR: Hyperfibrinogenemia
**Increased**
121
-Large pigmented protein that gives red cells their color
**Hemoglobin**
122
Components of hemoglobin | (3)
**-Protoporphyrin Ring IX** **-Tetramer of four polypeptide chains** **-Ferrous iron**
123
Function: -Carries oxygen to tissue and brings CO2 back
**Hemoglobin**
124
Reference method for measuring hemoglobin
**Cyanmethemoglobin**
125
Measures all forms of hemoglobin except for sulfhemoglobin
**Cyanmethemoglobin**
126
This form of hemoglobin can occur from antibiotics or pesticides
**Sulfhemoglobin**
127
TRUE or FALSE When measuring hemoglobin, Fe3+ is oxidized to Fe2+ by potassium ferricyanide to methemoglobin
**FALSE** **Fe2+ (ferrous iron) is oxidized to Fe3+ (ferric)**
128
Fe3+
**Ferric iron**
129
Fe2+
**Ferrous iron**
130
Normal reference range of hemoglobin for females
**12 - 15 g/dL**
131
Normal reference range of hemoglobin for males
**13.5 - 18 g/dL**
132
Normal reference range of hemoglobin for newborns
**16.5 - 21.5 g/dL**
133
Normal reference range of hemoglobin for children
**11 - 14 g/dL**
134
- Non-specific turbidity - High WBC - Lipemia - Hemoglobin C and S What do these do to hemoglobin measurement?
**Falsely elevate**
135
Definition: - Expressed as a % of RBC of the whole blood volume - Percentage of whole blood occupied by red cells after packing by centrifugation
**Hematocrit** **AKA** **Hct** **AKA** **PCV (packed cell volume)**
136
Calculation for automated hematocrit
Indirect Hct = RBC x MCV \_\_\_\_\_\_\_\_\_\_\_\_\_ 10
137
What is the Rule of Three?
**Hematocrit should be 3 times the hemoglobin +/- 3%** **Hct = 3 x Hgb**
138
Reference range for hematocrit for females
**35 - 49%**
139
Reference range for hematocrit for males
**40 - 54%**
140
Reference range for hematocrit for newborns
**48 - 68%**
141
Indice that tells us the size or volume of an RBC
**MCV** **-Higher the number, larger the cell**
142
TRUE or FALSE Agglutination can cause a falsely decreased MCV
**FALSE** **agglutination would falsely elevate the MCV**
143
In order to calculate MCV, you need to know what indices?
**RBC and Hct**
144
MCV calculation
**MCV (fL) =** **Hct x 10 \_\_\_\_\_\_\_\_\_\_\_** **RBC**
145
- Average weight of hemoglobin per red blood cell - Usually 1/3 of MCV - Measured in pg (picograms)
**Mean Cell Hemoglobin** **MCH**
146
Reference range for MCH
**26 - 34 pg**
147
Indices needed in order to calculate MCH
**Hemoglobin and RBC**
148
Calculation for MCH
**MCH (pg) =** **Hgb x 10 \_\_\_\_\_\_\_\_\_\_** **RBC**
149
How would hypochromasia affect MCH?
**Decreased cellular hemoglobin content**
150
How would hyperchromasia affect MCH?
**Increased cellular hemoglobin content**
151
What are 2 ways an MCH value could be altered?
**Lipemic specimen** **or** **Cold agglutinin**
152
- Indice that tells us the average hemoglobin concentration of each individual red blood cell in a sample - Usually corresponds with MCH
**MCHC** **Mean Cell Hemoglobin Concentration**
153
Reference range for MCHC
**32 - 36 %**
154
How would hypochromasia affect MCHC?
**Decreased MCHC**
155
How would hyperchromasia affect MCHC?
**Increased MCHC** **-too much hemoglobin for size of cell**
156
Indices needed in order to calculate MCHC
**Hemoglobin** **and** **Hematocrit**
157
Calculation for MCHC
**MCHC (%) =** **Hgb x 100 \_\_\_\_\_\_\_\_\_\_\_\_** **Hct**
158
Main function of RBCs
**-Carry oxygen to tissues** **-Bring CO2 back from tissues**
159
What part of an RBC is responsible for the transportation of O2 and CO2?
**Hemoglobin**
160
What part of the RBC is responsible for making the ATP to be used for energy?
**Mitochondria**
161
What do RBCs rely heavily on to produce ATP?
**Anaerobic glycolysis**
162
4 pathways used to provide ATP for red blood cells
**-Embden-Meyerhof Glycolytic Pathway** **-Hexose Monophosphate Shunt aka Phosphogluconate (Oxidative Pathway)** **-Methemoglobin Reductase Pathway** **-Rapaport-Leubering Shunt/Pathway**
163
This pathway provides RBCs with 90% of their ATP
**Embden-Meyerhof Glycolytic Pathway**
164
Pathway that maintains erythrocyte shape, flexibility, and membrane integrity
**Embden-Meyerhof Glycolytic Pathway**
165
Enzyme utilized in Embden-Meyerhof Glycolytic Pathway
**Pyruvate kinase**
166
This pathway provides RBCs 5-10% energy and is an oxidative pathway
**Hexose Monophosphate Shunt**
167
What enzyme does the Hexose Monophosphate Shunt utilize?
**Glucose-6-Phosphate-Dehydrogenase** **G6PD**
168
This pathway protect against oxidative injury from toxic reducing oxidants such as certain antimalarial drugs or infections
**Hexose Monophosphate Shunt**
169
If this pathway were defective, globin chains in the hemoglobin molecule would denature and precipitate as aggregates (Heinz bodies)
**Hexose Monophosphate Shunt**
170
This pathway is an "off-shoot" of Embden-Meyerhof -Maintains heme in the reduced ferrous (Fe3+)state which carries oxygen
**Methemoglobin Reductase Pathway**
171
Enzyme utilized in the Methemoglobin Reductase Pathway
**Methemoglobin reducatase**
172
If this pathway is defective/decreased, cyanosis occurs due to lack of oxygen binding
**Methemoglobin Reductase Pathway**
173
This pathway uses 1 ATP and produces 2,3-DPG (aka 2,3-BPG) which affects the oxygen affinity of hemoglobin
**Rapaport-Leubering Shunt/Pathway**
174
Enzymes utilized in the Rapaport-Leubering Pathway
**Biphosphoglycerate mutase** **and** **Biphosphoglycerate kinase**
175
Composition of RBC
**52% protein** **40% lipid** **8% carbohydrate**
176
What is responsible for providing strength to the lipid bilayer of an RBC?
**Cholesterol**
177
TRUE or FALSE Cholesterol and lecithin affect the surface area of RBCs which in turn affect their ability to carry oxygen
**TRUE**
178
These determine a red blood cells fluidity by maintaining differences in osmotic pressure, cation concentrations, and gas concentrations between external plasma and cytoplasm
**Phospholipids**
179
Glycolipids embedded in the membrane that carry several important RBC antigens (A, B, H, and Lewis - blood bank)
**Lipid matrix/ Phospholipids**
180
Predominant cytoskeletal (peripheral) protein in erythrocytes
**Spectrin**
181
Protein that extends from outer surface to inner side of erythrocytes
**Integral proteins**
182
Major glycoprotein that accounts for most of the sialic acid that gives erythrocytes their negative charge
**Glycophorin**
183
Proteins that are limited to the cytoplasmic surface of erythrocytes
**Peripheral proteins**
184
Major peripheral protein that: - Binds to other peripheral proteins and integral proteins - Forms skeleton and strengthens membrane to protect against shear forces
**Spectrin**
185
TRUE or FALSE The peripheral and integral proteins network for erythrocytes requires ATP
**TRUE**
186
Permeability of RBCs: Intracellular- to - extracellular ratio for sodium
1 : 12 1 sodium inside for ever 12 outside
187
Permeability of RBCs: Intracellular - to - extracellular ratio for potassium
**25 : 1** **25 potassium inside for every 1 outside**
188
Increased amounts of this ion will cause cell rigidity, shrunken cells with membrane protrusions (echinocytes) and reduced deformability
**Calcium**
189
Purpose of sialic acid found on the surface of glycophorin A of red blood cell membrane
**Produces a negative cell charge that causes the cells to repel each other (zeta potential)**
190
Molecule consisting of four globin protein chains containing a heme component nestled in a hydrophobic crevice
**Hemoglobin**
191
A tetrapyrrole ring with ferrous iron in the center
**Heme**
192
What percent of hemoglobin synthesis occurs during the nucleated stages of RBC maturation?
**65%**
193
What percent of hemoglobin synthesis occurs during the reticulocyte stage of RBC maturation?
**35%**
194
Tetramer of two pairs of unlike polypeptide chains
**Globin**
195
How many globin chains does a hemoglobin molecule have?
**4 chains** **-each with its own heme group**
196
How many groups of heme are in a hemoglobin molecule?
**4** **-Each containing a protoporphyrin ring plus ferrous iron (Fe2+)**
197
Most common disease caused by defective hemoglobin development
**Iron deficiency anemia (lack of iron)**
198
Diseases caused by defective hemoglobin (3)
**-Thalassemia (defective globin chains)** **-IDA (lack of iron)** **-Sideroblastic anemia (lack of heme)**
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\_\_/\_\_ of the body's iron is bound to heme
**2/3**
200
How much iron is needed daily for erythropoiesis?
**20 - 25 mg**
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\_\_/\_\_ of total body iron content is present as hemoglobin
**2/3**
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\_\_/\_\_ of total body iron content is present as tissue iron
**1/3**
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90% of tissue iron is storage iron known as?
**Ferritin**
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- Partially degraded ferritin - Less metabolically available than ferritin
**Hemosiderin**
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Where is hemosiderin seen?
**Liver** **Spleen** **Bone marrow**
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When is hemosiderin increased?
**Hemachromatosis** **and** **Iron Overload**
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What percent of tissue iron is non-available?
**10%**
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Iron that is lost each day, is replaced by the diet through which organ?
**Intestines**
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What is the average blood volume for a healthy adult?
**4500 - 5000 mL**
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Foods high in iron (4)
**-Organ meats** **-Wheat germ** **-Brewer's yeast** **-Legumes**
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Foods with a moderate level of iron (6)
**-Muscle meats** **-Fish** **-Fowl** **-Prunes** **-Some green vegetables (spinach)** **-Cereals (fortified)**
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Low molecular weight compounds that can increase iron absorption (3)
**-Amino acids** **-Fructose** **-Ascorbic acid (Vitamin C)**
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What decreases absorption of iron? (2)
**-Phosphates** **-Milk products (contains calcium phosphate)** **Ex. Antacids**
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What populations are most prone to iron deficiency anemia?
**Women and children**
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Where in the intestine is iron absorbed?
**Duodenum of the jejunum**
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This part of the cell produces heme
**Mitochondria**
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What influences the enzymatic steps of heme synthesis?
**Erythropoietin and Vitamin B6**
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Porphyrin synthesis starts with what 2 components?
**Glycine and Succinyl Coenzyme A**
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Protoporphyrin synthesis: What does the combination of Glycine and Succinyl Coenzyme A form?
**Aminolevulinic acid (ALA)**
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Protoporphyrin synthesis: What does Aminolevulinic acid become?
**Porphobilinogen**
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Protoporphyrin synthesis: What does porphobilinogen become?
**Coproporphyrinogen**
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Protoporphyrin synthesis: What does Coproporphyrinogen become?
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Protoporphyrin synthesis from start to finish (be able to recognize)
**Glycine + Succinyl Coenzyme A → Aminolevulinic Acid (ALA) → Porphobilinogen → Coproporphyrinogen → Protoporphyrinogen IX**
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What does Protoporphyrinuria cause?
**Werewolf sundrome**
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Protoporphyrin IX + Ferrochelatase = ?
**Heme**
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Any block in the enzymatic pathway will result in the excess formation of a particular porphyrin. What is this referred to as?
**Porphyrias**
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Where will porphyrin accumulate in the body?
**Brain** **Liver** **Bone marrow** **(very toxic if in the brain)**
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Protoporphyrin synthesis impairment will result in the accumulation of iron in the cytoplasm in the form of ________ aggregates
**Ferritin**
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Protoporphyrin synthesis impairment can cause the mitochondria to become encrusted with iron that will be visible around the nucleus of the RBC precursor. If we saw these cells stained with Prussian Blue, what would they be called?
**Ringed sideroblast**
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Globin synthesis is directed by how many genetic loci per haploid genome?
**8**
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What are the 7 different types of globin chains?
**Zeta** **Epsilon** **Gamma-A** **Gamma-G** **Delta** **Beta** **Alpha**
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**Alpha**
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**Beta**
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**Delta**
235
**Epsilon**
236
**Gamma** **-Remember Gamma has A and G**
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**Zeta**
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Which globin-like genes are on chromosome 16?
**Zeta** **Alpha-1** **Alpha-2**
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Which globin-like genes are on chromosome 11?
**Epsilon** **Gamma-G** **Gamma-A** **Delta** **Beta**
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Which globin genes are considered embryonic?
**Zeta** **Gamma** **Epsilon**
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These globin chains are produced up to 3 months following conception
**Zeta and Epsilon**
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Embryonic Hemoglobins: Zeta2, Epsilon2
**Hemoglobin Gower 1**
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Embryonic Hemoglobins: Alpha2, Epsilon2
**Hemoglobin Gower 2**
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Embryonic Hemoglobins: Zeta2, Gamma2
**Hemoglobin Portland**
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Embryonic Hemoglobins: Hemoglobin Portland
**Zeta2, Gamma2**
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Embryonic Hemoglobins: Hemoglobin Gower 2
**Alpha2, Epsilon2**
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Embryonic Hemoglobins: Hemoglobin Gower 1
**Zeta2, Epsilon2**
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Fetal hemoglobin contains which globin chains?
**Alpha2, Gamma2**
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This hemoglobin type has a high affinity for oxygen and by age 2 is less than 2% of total hemoglobin
**Hemoglobin F** **(Fetal hemoglobin)**
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When does Beta chain production reach adult percentages?
**Between 3-6 months postnatally**
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Make up of normal adult hemoglobins
**2 Alpha chains** **2 non-Alpha chains - Beta, Delta, or Gamma** **Hydrogen bonds** **Salt bridges**
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Polypeptide for Hemoglobin A
**Alpha2, Beta2**
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Alpha2, Beta2 makes what hemoglobin?
**Hemoglobin A**
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Polypeptide for Hemoglobin A2
**Alpha2, Delta2**
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Alpha2, Delta2 forms which hemoglobin?
**Hemoglobin A2**
256
Polypeptide for Hemoglobin F
**Alpha2, Gamma2**
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Alpha2, Gamma2 forms which hemoglobin?
**Hemoglobin F** **AKA** **Fetal Hemoglobin**
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Normal % of Hemoglobin A in adults
**\>95%**
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Normal % of Hemoglobin A in newborn - 2 years?
**10 - 40 %**
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Normal % of Hemoglobin A2 in adults
**\< 3.5%**
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Normal % of Hemoglobin A2 in newborns
**\< 1%**
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Normal % of Hemoglobin F in adults
**1 - 2%**
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Normal % of Hemoglobin F in newborn to 2 years
**60 - 90%**
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At birth, Hemoglobin F levels are what %?
**60 - 90%**
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At 12 weeks, Hemoglobin F is at what %?
**7%**
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Between 4-5 months, Hemoglobin F reaches what %?
**1.1 - 5.3%**
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As an adult, Hemoglobin F is found at what %?
**1 - 2%**
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This comprises 33% of an RBC
**Hemoglobin**
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One of the most important controls of hemoglobin affinity for oxygen is which RBC organic phosphate?
**2,3-diphosphoglycerate** **AKA** **2,3-biphosphoglycerate**
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What does 2,3-DPG (2,3-BPG) do when part of the hemoglobin molecule?
**Widens the space between beta chains, forming an anionic salt bridge and creating deoxyhemoglobin**
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What happens to the hemoglobin molecule when 2,3-DPG (2,3-BPG) is absent?
**The salt bridge is broken and the hemoglobin molecule will carry more oxygen - creating oxyhemoglobin**
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Location that oxyhemoglobin occurs
**In the lungs**
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Amount of iron needed each day to replace iron lost by senescent RBCs
**18.5 - 21 mg**
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TRUE or FALSE Increased oxygen affinity means the hemoglobin more readily gives up the oxygen
**FALSE** **Increased oxygen affinity means the hemoglobin does NOT readily give up oxygen**
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TRUE or FALSE Decreased oxygen affinity means hemoglobin releases oxygen more readily (hemoglobin has a low affinity for oxygen)
**TRUE**
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If the Hemoglobin-Oxygen Dissociation Curve shows a more BASIC pH, it is noted as what type of shift?
**Left Shift**
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If the Hemoglobin-Oxygen Dissociation Curve shows a more ACIDIC pH, it is noted as what type of shift?
**Right shift**
278
How is oxygen tension measured?
**mmHg** **millimeters of Mercury**
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TRUE or FALSE Oxygen affinity being decreased causes more oxygen to be released to the tissues. This causes a right shift.
**TRUE**
280
What causes a right shift in the hemoglobin-oxygen dissociation curve?
**Hypoxia** **Increased 2,3-DPG** **Anemia** **Acidosis** **Fever**
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TRUE or FALSE Oxygen affinity being increased causes decreased amounts of oxygen being delivered to tissues. This causes a left shift.
**TRUE**
282
TRUE or FALSE An increase in oxygen affinity causes a decrease in oxygen delivery
**TRUE**
283
What causes a left shift on the hemoglobin-oxygen dissociation curve?
**Decreased 2,3-BPG** **Alkalosis** **Increased quantity of abnormal hemoglobin** **Multiple transfusions (new blood/not as oxygenated)** **Fetal hemoglobin (higher affinity for oxygen, so holds on to it)**
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Definition: Inadequate amount of oxygen at tissue level
**Hypoxia**
285
Definition: Bluish color of skin due to presence of high amounts of deoxyhemoglobin in blood
**Cyanosis**
286
3 acquired nonfunctional hemoglobins
**Carboxyhemoglobin** **Methemoglobin** **Sulfhemoglobin**
287
This acquired nonfunctional hemoglobins oxygen affinity is 200x greater than that of normal hemoglobin. It can be fatal. Smokers can have up to 12%
**Carboxyhemoglobin**
288
This nonfunctional hemoglobin is incapable of combining with oxygen due to the iron being in the ferric state. Increased levels are formed when exposed to certain oxidizing drugs or chemicals. Infants are more susceptible due to HbF more readily converting to it.
**Methemoglobin**
289
This nonfunctional hemoglobin is irreversible
**Sulfhemoglobin**
290
This returns iron to bone marrow or liver
**Transferrin**
291
What is the protoporphyrin ring broken down to?
**Toxic bilirubin**
292
What carries toxic bilirubin from the breakdown of the protoporphyrin ring to the liver?
**Albumin**
293
What % of aged erythrocyte destruction occurs extravascularly?
**90%**
294
What % of erythrocyte destruction occurs intravascularly?
**10%**
295
Approximately what % of the total red cells are removed from circulation on a daily basis?
**~1%**
296
TRUE or FALSE Oxygen affinity is dependent on 2,3-BPG
**TRUE**
297
When are enzymes made in RBCs
**When RBCs still have a nucleus**
298
Size of reticulocytes
**9 microns**
299
Purpose of haptoglobin
**Prevents kidney damage by binding hemoglobin dimers after intravascular hemolysis occurs**
300
Hemoglobin synthesis occurs here
**Nucleated red blood cells in the bone marrow**
301
What is involved in protein synthesis?
**Transcription** **Translation** **and Codons**
302
Automated instruments use what indices to calculate hematocrit?
**MCV and RBC**
303
What is urobilinogen the final breakdown product of?
**Hemoglobin**
304
This abnormal RBC shape has an MCHC \>36
**Spherocytes**
305
Iron is stored as \_\_\_\_\_\_\_
**Ferritin**
306
Iron is carried by __________ to the liver and bone marrow
**Transferrin** **(b/c trans = across or to the other side)**
307
Defective hemoglobin synthesis causes this disease
**Porphyria**
308
Rule of 3
**Hemoglobin x 3** **+/-3** **= Hematocrit**
309
Normal RDW range
**11.5 - 14.5%**
310