1
Q
If a patient were suffering from appendicitis, you would expect which cells to be increased in the peripheral blood?
A
neutrophils
2
Q
Which cells make up the biggest mass of cells in the peripheral blood?
A
erythrocytes
3
Q
These cells are important in maintaining normal hemostasis.
A
platelets
4
Q
A patient has a WBC count of 25 x 10%/L. The doctor subsequently orders a differential count. This is an example of
A
reflex testing
5
Q
The pre-examination component of laboratory testing includes:
A
storage, collection
6
Q
A decrease in erythrocytes is accompanied by a decrease in:
A
hemoglobin (and hematocrit)
7
Q
Laboratory screening tests include all of the following except:
A. bone marrow examination
B. WBC count
C. prothrombin time
D. RBC indices
A
bone marrow examination
8
Q
An increase in the concentration of neutrophils in the peripheral blood is most commonly due to:
A
infection
9
Q
An upset in the balance of coagulation proteins can result in a defect of:
A
hemostasis
10
Q
Apoptosis is best described as:
A
programmed cell death
11
Q
What are considered to be initiators of apoptosis?
A
growth factor withdrawal
loss of attachment to extracellular matrix
cell-damaging stress
(A. growth factor withdrawal, B. loss of attachment to extracellular matrix, C. cell-damaging stress)
12
Q
All of the following are events regulated by apoptosis EXCEPT:
A. elimination of auto reactive lymphocytes
B. elimination of expanded lymphocytes following cessation of immune response
C. elimination of expanded erythrocytes following cessation of hypoxic episode
D. elimination of expanded phagocytic cells following cessation of infection/inflammatory response
A
elimination of expanded erythrocytes following cessation of hypoxic episode
13
Q
The proteosome is:
A
a protease complex used for destruction of ubiquitin-labeled molecules
14
Q
The cell-cycle regulatory protein that functions to inhibit cell cycling by sequestering transcription factors required for cell-cycle progression from G1/S is:
A
Rb
15
Q
The protein responsible for monitoring DNA damage and the integrity of the genome is:
A
p53
16
Q
All of the following are considered diseases associated with increased apoptosis EXCEPT:
A. aplastic anemia
B. AIDS
C. Parkinson’s disease
D. systemic lupus erythematosus
E. toxic induced liver disease
A
D. systemic lupus erythematosus
Also: cancers, other autoimmune disorders and viral infections
17
Q
How would you differentiate between basophilic stippling and Pappenheimer bodies in a Wright’s-stained blood smear?
A
B. Stain another blood smear with Prussian blue
Pappenheimer bodies are identified with prussian blue
18
Q
How can basophilic stippling be differentiated from polychromasia?
A
Basophilic stippling stains as blue-black granules whereas polychromasia stains a diffuse pinkish-gray on the Wright’s stain
19
Q
Upon review of a stained blood smear you note that erythrocytes appear to form irregular clusters, like grapes. How would you report this?
A
RBC agglutination noted
20
Q
In a child with hemoglobin M, what would you expect to find?
A
A leftward shift on the ODC
- methemoglobin is ferric (Fe3+) which cannot bind oxygen, so the other heme groups bind it more tightly as a result, and they do not want to give it up to the tissues, causing hypoxia
21
Q
What do decreased haptoglobin and hemopexin levels indicate?
A
increased intravascular RBC destruction
22
Q
Which statement about RBC catabolism and hemoglobin dissociation is NOT true?
A. Released iron is transported in plasma by haptoglobin back to the marrow.
B. Globin is catabolized to amino acids that enter the amino acid pool.
C. Porphyrin rings are broken, producing carbon monoxide and biliverdin.
D. Bilirubin is carried to the liver, conjugated, and excreted in the bile.
A
A. Released iron is transported in plasma by haptoglobin back to the marrow
23
Q
In which cellular location does the insertion of iron into the protoporphyrin ring take place?
A
mitochondria
24
Q
When should hemoglobin electrophoresis be considered?
A
methemoglobinemia
25
What is the best explanation for a patient with hemoglobin of 9 g/dL but no symptoms of anemia due to chronic intestinal bleeding?
his ODC is shifted right
26
Why does long-term O2 therapy in a patient with emphysema increase the amount of O2 delivered to tissues?
It increases the PO2 of inspired air, therefore increasing saturation of hemoglobin with O2
27
What effect does the release of O2 in the capillaries have on the H+ concentration in tissues?
Decreases it
28
What is the best explanation for rapid heart rate and fast breathing at 8,000 feet above sea level?
The atmospheric PO2 is decreased, which means a shift to the left will need to happen in order to pick up more O2 in the lungs (but can lead to tissue hypoxia)
29
In iron deficiency, what would you expect to occur?
decreased ALAS activity
30
An increase in the concentration of HbA1c is an indication of:
diabetes
31
Which of the following shifts the ODC to the right?
A. decreased PCO2
B. increased PO2
C. decreased H+ concentration
D. increased temperature
increased temperature
32
Which of the following hemoglobins is the predominant hemoglobin in infants?
hemoglobin F
33
Normally, hemoglobin concentration is maintained in a steady state by:
balance in the production and destruction of erythrocytes
34
In extravascular hemoglobin destruction pathways the alpha-methane bridge of the porphyrin is cleaved, producing a molecule of what?
Carbon monoxide
35
Hemosiderinuria can be confirmed and visualized with which of the following tests?
Prussian blue stain of urine sediment (stains the iron in hemoglobin in the urine)
36
All of the following are by-products of heme catabolism EXCEPT:
A. carbon monoxide
B. hemosiderin
C. bilirubin
D. haptoglobin
haptoglobin
37
Which of the following is the major hemoglobin found in adults?
Hemoglobin A- two alpha chains and two beta chains
38
Heinz bodies are the result of which of the following?
C. precipitated hemoglobin
39
Which of the following is the predicted result of increased EPO levels?
A. hemoglobin with a low oxygen affinity
B. bone marrow hypoplasia
C. decreased number of sideroblasts
D. normoblastic hyperplasia
D. normoblastic hyperplasia
40
Deduce which of the following is a cause of abnormality in cell shape.
A. deficiency in Rapoport-Leubering pathway
B. abnormal integral proteins
C. abnormal peripheral proteins
D. deficiency of methemoglobin reductase
abnormal peripheral proteins
41
The oligosaccharide chains on the surface of the RBC membrane have all the following functions, EXCEPT:
A. comprise the ABO antigens
B. determine the shape of the RBC
C. provide a negative surface charge
D. form part of the integral proteins
B. determine the shape of the RBC
42
Which of the following is a minor hemoglobin component in an adult?A. hemoglobin A
B. hemoglobin A2
C. hemoglobin Portland
D. hemoglobin Gower I
B. Hemoglobin A2
43
What effect would iron deficiency have on the rate of hemoglobin synthesis?
decrease it
44
When 2,3-BPG increases, the oxygen affinity of hemoglobin:
decreases
45
How many iron molecules are present in a molecule of hemoglobin?
four
46
The body’s most common and efficient method of removal of aged or abnormal erythrocytes, and recovery of essential components such as iron and amino acids, is
Extravascular erythrocyte destruction
47
Which of the following describes the reticulocyte?
A. contains RNA
B. reference range is 5 to 15%
C. has a pyknotic nucleus
D. is smaller than a mature RBC
contains RNA
48
When hemoglobin binds 2,3-BPG in the erythrocyte, it has which effect?
oxygen is released from hemoglobin, the affinity decreases
49
The presence of echinocytes in the peripheral blood may be an indication of
accumulation of calcium in the cell
50
Which of the following statements about reticulocytes is false?
A. They are the last nucleated stage of erythrocyte maturation.
B. They have a normal average circulating period of one day.
C. They contain cytoplasmic RNA.
D. They are still actively synthesizing hemoglobin.
A. they are the last nucleated stage of rbc maturation
51
Erythrocyte survival depends on an adequate supply of ATP for the following reason:
to maintain cell flexibility and shape
52
The elastic extension ability of erythrocytes is predominantly due to the
coiled spectrin tetramers
53
Which of the following stages in erythrocyte development can be found in both the bone marrow and peripheral blood?
A. reticulocyte
B. pronormoblast
C. polychromatophilic normoblast
D. orthochromatic normoblast
reticulocyte
54
An NRBC has the following characteristics: no visible nucleoli, moderate N:C ratio, deeply basophilic cytoplasm, and irregular clumping of chromatin on the nuclear rim. These characteristics describe which of the following maturation stages?
A. orthochromic normoblast
B. basophilic normoblast
C. pronormoblast
D. metarubricyte
basophilic normoblast
55
The majority (90%) of the erythrocyte's metabolism for energy production is produced from
glucose, in the glycolytic pathway
56
Which erythroid maturation stage would be the most sensitive to erythropoietin stimulation?
CFU-E
57
The erythrocyte membrane is important in all of the following except:
- maintaining erythrocyte flexibility
- generation of ATP
- maintaining erythrocyte shape
- carrier for cell antigens
generation of ATP
58
Which of the following describes normal erythrocytes?
- contain iron granules
- cell volume between 70-100 fL
- mean diameter of 7-8 mcM
- Life span of 90 days
mean diameter of 7-8 microns
59
In extravascular hemoglobin destruction, heme rings are broken down with the resulting direct production of
Carbon monoxide
60
This refers to the localized environment in the bone marrow that is crucial for the development of hematopoietic cells
hematopoietic microenvironment
61
This type of signal is produced and secreted by one cell and acts on a nearby cell
paracrine
62
What are the major hematopoietic sites in the fetus and in the adult?
liver (fetus) bone marrow (adult)
63
How do cells get from the bone marrow into peripheral circulation?
Mature cells squeeze through spaces in the vessel endothelial lining
64
Enlarged lymph nodes are commonly found in all of the following except:
- viral infections
- liver cirrhosis
- bacterial infections
- metastatic malignancy
liver cirrhosis
65
Using Wright stain, what is a feature of normal cells as they mature?
nuclear chromatin becomes more condensed
66
Kupffer cells, found in the liver, are
macrophages
67
What hematopoietic precursor cells have restricted developmental potential, cannot self renew, and are not morphologically recognizable
progenitor cells
68
The proliferation and differentiation responses of a progenitor cell are induced by
binding of cytokines to cell receptors
69
Hereditary spherocytosis may lead to splenomegaly because of
A. increased lymphoid tissue
B. increased removal of erythrocytes
C. the accumulation of undigestible substances
D. the presence of tumor cells
B. increased removal of erythrocytes
70
Differentiation is best described as
A. the appearance of different properties in cells that were initially equivalent
B. the instance when two cells, derived from the same precursor, take separate routes of development
C. the totality of phenomena that begins with commitment and ends when the cell has all of its
characteristics
D. the “aging” or senescence of a cell
A. the appearance of different properties in cells that were initially equivalent
71
The morphologically recognizable population of hematopoietic precursor cells, capable of amplification by proliferation, are the
maturing cells
72
Hematopoietic growth factors exert all of the following effects on hematopoietic cells except
A. promote cell survival by suppressing apoptosis
B. promote cell proliferation
C. regulate the processes of differentiation
D. promote senescence
promote senescence
73
All of the following are characteristics of hematopoietic growth factors EXCEPT:
A. most growth factors are produced by stromal cells in the microenvironment
B. individual growth factors, by themselves, are poor stimulators of colony growth
C. most growth factors are relatively lineage-specific
D. growth factors commonly act synergistically with other cytokines
C. most growth factors are relatively lineage-specific
74
Describes the instance when two cells derived from the same precursor take a separate route of development
commitment
75
These hematopoietic precursor cells give rise to all bone marrow cells by proliferation and differentiation
stem cells
76
The CFU-GEMM is an example of what kind of precursor cell
multipotential progenitor cells
77
What is an example of a late acting growth factor
erythropoietin
78
What is the correct sequence of location of hematopoiesis during development
yolk sac, AGM, liver, bone marrow
79
What cell types are found in the bone marrow?
- adipocytes
- osteoblasts
- osteoclasts
- macrophages
80
Which of the following is true of the thymus?
- It enlarges after adolescence
- It provides mature macrophages to the rest of the body
- It has a high rate of cell death
- It is nonfunctional in old age
it has a hight rate of cell death
81
Which region of the spleen would most likely be normal in an immune-deficient person?
red pulp
82
Both lymph node and spleen have which structure?
B cell follicle
83
Bone marrow hyperplasia occurs most commonly in association with
ineffective erythropoiesis
84
What causes hypersplenism?
What is an example of something that does not?
causes: cirrhosis, antibody coated red cells, metastatic tumor
does not: clot in splenic artery
85
What is a common cause of lymphadenopathy
infection
86
What may occur after splenectomy for blood disorders associated with antibodies directed against red blood cells?
increased risk of infection
87
What is the correct order for the steps of making heme?
ALAS
- porphobilinogen
- uroporphyrinogen
- coproporphyrin
- protoporphyrin
- heme
por ur cop proto heme
88
In hemoglobin, the iron atom is found in side the
protoporphyrin ring
89
What hemoglobin has the highest affinity for oxygen?
Hemoglobin F (fetal hemoglobin)
90
Name the cells
acanthocytes
91
Name the cells
Degmacytes (bite cells)
92
Name the cells
blister cells
93
Name the cells
burr cells
94
Name the cells
echinocytes (crenated)
95
Name the cells
elliptocytes
96
Name the cells
Helmet cells
97
Name the cells
Keratocytes
98
Name the cells
Knizocytes
99
Name the cells
Leptocytes
100
Name the cells
ovalocytes
101
Name the cells
Pyknocytes
102
Name the cells
schistocytes
103
Name the cells
drepanocytes (sickle cells)
104
Name the cells
Spherocytes
105
Name the cells
stomatocytes
106
Name the cells
target cells
107
Name the cells
teardrop (decryocytes)
108
Name the cells
hypochromic
109
What cells are the same, but called different names when using different stains?
Polychromatic cells and reticulocytes
110
The last stage in erythrocyte development that contains a nucleus is called
orthochromic normoblast
111
The presence of hemosiderinuria infers which of the following?
increased intravascular hemolysis
112
To stain and count reticulocytes, what stain must be used?
A supravital stain: New methylene blue or brilliant cresyl blue
113
What is the inclusion in reticulocytes?
RNA
114
The presence of Heinz bodies is an indication of a defect in the
hexose monophosphate shunt
115
When 2,3 BPG increases, the oxygen affinity of hemoglobin
decreases
116
A decrease in 2,3 BPG does what to the oxygen affinity of hemoglobin?
increases it
117
Compared to the later stages, cells in the early BFU-E stage of RBC formation are known for being
relatively insensitive to EPO
118
Name the inclusion (and what it means)
basophilic stippling- granules composed of ribosomes and RNA
- defective/ accelerated heme synthesis
- lead poisoning
- severe anemias
119
Name the inclusion (and what it means)
Cabot rings- nuclear remnants, abnormal histone synthesis
- lead poisoning
- pernicious anemias
120
Name the inclusion (and what it means)
Heinz bodies- denatured (oxidized) precipitated hemoglobin
- the hexose monophosphate shunt defective
- G6PD deficiency
- oxidizing drug use like phenacetin, sulfa antibiotics
121
What stain must be used to identify Heinz bodies?
a supravital stain: crystal violet or brillant cresyl blue
122
Name the inclusion (and what it means)
Howell Jolly bodies
- accelerated or abnormal erythropoiesis (spleen cannot keep up with pitting remnants from the cell)
- post splenectomy, atrophy of the spleen
123
Name the inclusion (and what it means)
Pappenheimer bodies/siderotic granules
- aggregates of mitochondria, ribosomes, iron
- ferric ions appear blue
124
What stain needs to be used for Pappenheimer bodies?
prussian blue
125
What is wrong with the iron in methemoglobin?
It has been oxidized and can no longer carry oxygen
126
Ferric versus ferrous
Ferric 3+ - body cannot use
Ferrous 2+ - body can use
127
If you see several RBCs on a peripheral smear that are larger than normal and show polychromasia, what does this indicate?
- the body is actively making many new RBCs
- lacks normal levels of hemoglobin
- would also show a high retic count
128
Where is erythropoietin produced?
The kidneys
129
20. An increase in erythrocyte surface area can be directly correlated with which of the following?
A. an increase in blood urea nitrogen
B. an increase in free fatty acids
C. an increase in plasma cholesterol
D. a decrease in spectrin
an increase in plasma cholesterol
130
What are senescent cells? What happens to them?
declining cells, they are destroyed in the liver, spleen, and bone marrow by macrophages
131
Which cells are the iron recyclers for the body?
macrophages
132
What is BFU- E
- burst forming unit
- gives rise to many hemoglobin containing RBC precursors "burst" out
- gives rise to CFU-E
- not recognizable
133
Does BFU-E or CFU-E have CD34?
BFU has CD 34, it can still make self copies (stem cell) and is not recognizable
CFU loses CD 34 as it matures
134
CFU-E
- colony forming unit
- immediate precursor of pronormoblasts
- still not recognizable
135
What is sensitive to EPO, BFU-E or CFU-E?
BFU is not sensitive
CFU is sensitive
136
Are BFU-E and CFU-E committed progenitor cells?
Yes
137
What is another name for erythroblasts?
normoblasts
138
Do reticulocytes have a nucleus?
No, only residual RNA
139
What are erythroblasts?
- all the nucleated precursors in the bone marrow
- morphologically identifiable
140
What are the six stages of normoblastic maturation?
1. Pronormoblast
2. Basophilic normoblast
3. Polychromatic normoblast
4. Orthochromatic normoblast (nRBC)
5. Reticulocyte (polychromatic erythrocyte, no nucleus)
6. Erythrocyte
PB PORE
141
Every stage of maturation from stem cell to final mature RBC (9)
1. HSC
2. Common myeloid progenitor
3. Megakaryocyte- Erythrocyte progenitor
4. Pronormoblast
5. basophilic normoblast
5. polychromatic normoblast
6. orthochromatic normoblast
7. reticulocyte (polychromatic erythrocyte)
8. erythrocyte
142
What is the earliest recognizable RBC precursor?
pronormoblast (first in maturation stage)
143
What is the N:C ratio in maturing RBCs?
starts high and gets lower until there is no nucleus
pronormoblast has highest N:C ratio
144
What key things are in a pronormoblast that make it identifiable?
- very large nucleus (80% of cell)
- nucleoli
- light golgi halo on the side of the nucleus
145
Whey key things can help to differentiate a pronormoblast from a basophilic normblast?
- basophilic have a granular nucleus (pronormoblast is smoother)
- basophilic do not have nucleoli
- basophilic have a smaller N:C ratio than pronormoblasts
146
Why is the nucleus of the basohphilic normoblast more granular than in the pronormoblast?
- basohphilic has courser chromatin (more heterochromatin, more mature)
- pronormo has more euchromatin
147
How does the polychromatic normoblast appear when stained?
The cytoplasm is grey/blue, lighter than in the previous stages and bigger (lower N:C ratio)
148
Which cells are the NRBCs most often seen in the peripheral blood?
orthochromic normoblast
149
What stage of RBC maturation is the last with a nucleus?
orthochromic normoblast
150
How do orthochromic normoblasts appear when stained?
- smaller, closer to normal RBC size
- pink cytoplasm
- dark circular nucleus that stands out
151
What does a prussian blue stain identify?
Iron- aka pappenheimer bodies in the cell
152
Siderocytes vs sideroblasts
- siderocytes are RBCs with identified iron
- sideroblasts are nucleated RBCs with iron in the cytoplasm
153
Erythroblastic islands
- in the bone marrow
- a central macrophage surrounded by developing erythroblasts and reticulocytes
- macrophage sends out signals for development, will phagocytize the nucleus of the developing RBC and any defective cells
- when they are ready and mature, they detach and enter the circulation
154
Cytoskeleton of the RBC
provides them with the strength and flexibility they need to survive in circulation
155
What does the erythroblast membrane have receptors for?
EPO and transferrin
156
What cell morphology is the result of expansion of the inner leaflet of the PM due to high amounts of cholesterol?
stomatocytes
157
What cell morphology is the result of the expansion of the outer leaflet of the PM due to high amounts of cholesterol?
echinocytes (crenated)
158
What cell morphology is the result of the overall cholesterol: phospholipid ratio of the PM increasing?
acanthocytes
159
What part of the RBC membrane is essential for transport of proteins and anions?
integral proteins
160
What is the overall purpose of the glycolytic pathway?
production of ATP from glucose
161
How much of the RBC glucose is metabolized by the glycolytic pathway?
90-95%
162
How much of the RBC glucose is metabolized by the hexose monophosphate shunt?
5-10%
163
What does the hexose monophosphate shunt produce?
NADPH instead of ATP
164
What pathway protects hemoglobin from becoming oxidized?
hexose monophosphate shunt
165
What is necessary for maintaining hemoglobin in the in the reduced functional state?
GSH- glutathione
(in the HMP)
166
What is the first step of the HMP?
Glucose 6 phosphate is oxidized by glucose 6 phosphate dehydrogenase (G6PD)
167
What happens when the HMP shunt is defective?
- Hemoglobin denatures (is oxidized) and precipitates to form heinz bodies
168
What is the purpose of the methemoglobin reductase pathway? How does it work?
- maintains heme iron in the reduced functional ferrous state (fe2+)
- MetHb reductase and NADH reduce Fe3+ to Fe 2+
- also prevents hemoglobin from being oxidized
169
methemoglobin
- hemoglobin with iron in the ferric state (Fe3+)
- generated when O2 dissociates from heme iron
- cannot bind O2
170
What can interfere with the MetHb reductase?
some oxidant drugs
171
What happens when the methemoglobin reductase pathway is defective?
- cyanosis
- high levels of MetHb
- Shift to the left on the ODC
172
What pathway needs ATP?
The Rapoport Leubering Shunt sacrifices one of the 2 ATP molecules made during glycolysis
173
What does the R-L shunt regulate?
The delivery of oxygen to tissues
- produces 2,3 BPG which decreases affinity for O2 in Hg and O2 can be released to tissues
174
What does the relaxed state of hemoglobin mean?
- oxyhemoglobin
- oxygen is bound, there is a high affinity
- indicates a shift to the left
175
What does the tense state of hemoglobin mean?
- deoxyhemoglobin
- oxygen is not bound, there is a decreased affinity due to 2,3 BPG
- indicates a shift to the right
176
Why does fetal hemoglobin have a high affinity for oxygen, what shift is this?
in utero is a hypoxic environment, so there is a high affinity for O2
this is a shift to the left
177
At higher altitudes, what kind of shift is there, why?
At higher altitudes there is a higher affinity for oxygen, because the partial pressure of oxygen is lower
this is a shift to the left
178
When does EPO increase?
- impaired delivery of O2 to tissues (hypoxia)
- low partial pressure of O2
179
Where does extravascular destruction take place?
mostly in macrophages of spleen and liver
180
RBC destruction is usually the result of
senescence
181
What two plasma proteins will decrease in response to intravascular hemolysis?
- haptoglobin
- hemopexin
182
What is the primary cause/effector of erythropoiesis?
tissue hypoxia
183
What is necessary to maintain reduced levels of methemoglobin?
NADH
184
Is the iron from a senescent cell recycled in the body?
yes
185
Rule of 3
hemoglobin x 3 = hematocrit +/- 3
186
What could mess with the rule of 3?
Anything that could falsely elevate hemoglobin
- turbidity
- high white count
- lipemic
- RBCs containing Hb C or S
Agglutination and rouleaux can falsely increase HCT
187
What can falsely decrease ESR?
- sickle cells
- rouleaux
- cold agglutination
188
What are adult hemoglobin composed of?
two alpha and two beta chains
189
What are fetal hemoglobin composed of?
two alpha and two gamma chains
190
Primary lymphoid tissues
bone marrow thymus
191
Secondary lymphoid tissues
spleen and lymph nodes
192
Hematopoietic tissues in adults
bone marrow
thymus
spleen
lymph nodes
193
Causes of left shifts
- hypocarbia
- hyperventilation
- oxygen toxicity
- low temp
- fetal hemoglobin
194
Causes of right shifts
- muscles
- placenta
- hypercarbia
- fever
- anemia
Hematology
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