WorksheetsHEMA-4
Total questions: 115
Worksheet time: 58mins
Name
Class
Date
1.
Which of the following exhibits a mutation in the band 3 protein?
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
2.
Deficient protein: Ankyrin, Band 3, alpha spectrin, beta spectrin, and protein 4.2
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
3.
Which of the following is autosomal recessive?
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
4.
The following are autosomal dominant except:
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
5.
Patients with this conditions have an increase in the MCHC value between 35 and 38 g/dL
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
6.
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)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
7.
Defect in proteins that disrupt the horizontal linkages in the protein cytoskeleton; loss of mechanical stability of membrane
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
8.
Severe defect in spectrin that disrupts horizontal linkages in protein cytoskeleton; severe RBC fragmentation
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
9.
Defect in band 3 causing increased membrane rigidity; resistant to malaria
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
10.
Which of the following is prevalent in some areas of Southeast Asia?
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
11.
Which of the following is a rare subtype of hereditary elliptocytosis?
a)
Hereditary spherocytosis
b)
Hereditary elliptocytosis
c)
Hereditary pyropoikilocytosis
d)
Southeast Asian Ovalocytosis
12.
Increased membrane permeability to sodium and potassium; increased intracellular sodium causing influx of water, increase in cell volume, and decreased cytoplasmic viscosity; typical RBC morphology: stomatocytes (5% to 50%) and macrocytes
a)
Overhydrated Hereditary Stomatocytosis
b)
Dehydrated Hereditary Stomatocytosis
13.
Which of the following is the most common form of stomatocytosis?
a)
Overhydrated Hereditary Stomatocytosis
b)
Dehydrated Hereditary Stomatocytosis
14.
Increased membrane permeability to potassium; decreased intracellular potassium, resulting in loss of water from cell, decrease in cell volume, and increased cytoplasmic viscosity; typical RBC morphology: target cells, burr cells, stomatocytes (<10%), RBCs with “puddled” hemoglobin at periphery, desiccated cells with spicules
a)
Overhydrated Hereditary Stomatocytosis
b)
Dehydrated Hereditary Stomatocytosis
15.
What are the deficient proteins in Hereditary elliptocytosis?
a)
alpha spectrin
b)
beta spectrin
c)
band 3
d)
protein 4.1
16.
What is the deficient protein in Southeast Asian Ovalocytosis?
a)
alpha spectrin
b)
beta spectrin
c)
band 3
d)
protein 4.1
17.
What is the deficient protein in overhydrated hereditary stomatocytosis?
a)
Rh-Associated protein
b)
Piezo-type mechanosensitive channel component 1
c)
Alpha spectrin
d)
Beta spectrin
18.
What is the deficient protein in dehydrated hereditary stomatocytosis?
a)
Rh-Associated protein
b)
Piezo-type mechanosensitive channel component 1
c)
Alpha spectrin
d)
Beta spectrin
19.
What are the deficient proteins in Hereditary pyropoikilocytosis?
a)
alpha spectrin
b)
beta spectrin
c)
band 3
d)
protein 4.1
20.
✓ The RBC count, hemoglobin and hematocrit values are ____ in people living at a HIGHER ALTITUDE over what they would be at sea level.
a)
Lower
b)
Higher
c)
Either
d)
Neither
21.
The difference in RBC count is around ___ at 2 km altitude
a)
1 g Hb/dL
b)
2 g Hb/dL
c)
3 g Hb/dL
d)
4 g Hb/dL
22.
The difference in RBC count is around ___ at 3 km altitude
a)
1 g Hb/dL
b)
2 g Hb/dL
c)
3 g Hb/dL
d)
4 g Hb/dL
23.
Glucose penetrates the red blood cell with no energy expenditure via Glut-1, a transmembrane protein
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
24.
Which of the following handles 90% of glucose utilization in red blood cells?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
25.
Which of the following is a non-oxidative, anaerobic pathway?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
26.
Which of the following is produces 2 molecules of ATP?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
27.
Which of the following is also known as pentose phosphate pathway?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
28.
Which of the following is also known as phosphogluconate pathway?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
29.
Which of the following pathway involves aerobic glucolysis?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
30.
Which of the following pathway aerobically converts glucose to pentose and produces NADPH (nicotinamide adenine dinucleotide phosphate reduced)?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
31.
In this pathway, NADPH reduces glutathione (reduced glutathione reduces peroxides and guards proteins, lipids, and heme iron from oxidation
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
32.
Which of the following pathways prevents denaturation of the globin by oxidation?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
33.
Which of the following pathways is functionally dependent on G6PD enzyme?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
34.
Which of the following pathways is also known as Cytochrome B5 reductase?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
35.
Which of the following pathways maintains iron in the heme in its reduced phase?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
36.
Which of the following pathways is used for the production of 2,3 BPG?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
37.
Which of the following pathways involves binding of 2,3-BPG to hemoglobin and decreases the oxygen affinity of hemoglobin?
a)
Embden-Meyerhof Pathway
b)
Hexose Monophosphate Shunt
c)
Methemoglobin Reductase Pathway
d)
Rapoport-Luebering Shunt
38.
Increased pH, decreased pCO2, 2-3 BPG, and temperature
a)
Shift to the left
b)
Shift to the right
39.
Decreased pH, Increased pCO2, 2-3 BPG, and temperature
a)
Shift to the left
b)
Shift to the right
40.
Decreased affinity
a)
Shift to the left
b)
Shift to the right
41.
Increased affinity
a)
Shift to the left
b)
Shift to the right
42.
a shift in the curve due to an alteration in pH (or hydrogen ion concentration)
a)
Bohr effect
b)
Haldane effect
43.
effect of hydrogen ions and CO2 on the affinity of hemoglobin for oxygen
a)
Bohr effect
b)
Haldane effect
44.
depicts the occurrence by which the binding of O2 to the hemoglobin promotes the release of CO2
a)
Bohr effect
b)
Haldane effect
45.
the curve produced when the 2 variables (partial pressure of oxygen and affinity of hemoglobin for oxygen) are plotted on a graph (oxygen saturation of hemoglobin versus the partial pressure of oxygen)
a)
Bell curve
b)
Oxygen dissociation curve
c)
Carbon dioxide dissociation curve
46.
The affinity of hemoglobin for oxygen is dependent on the following factors: EXCEPT
a)
pH
b)
Partial pressure of carbon dioxide
c)
Concentration of 2,3-biphosphoglycerate (2,3-BPG)
d)
Temperature
e)
Presence of other hemoglobin species that are functional
47.
used by the RBCs in the following ways:
✓ Maintenance of RBC shape and deformability
✓ Gives energy for the active transport of cations
✓ Helps in modulating the amount of 2,3-BPG generated
a)
BPG
b)
DPG
c)
ADP
d)
ATP
48.
Which of the following is an autosomal recessive disorder?
a)
Pyruvate Kinase (PK) Deficiency
b)
Glucose-6-phosphate-dehydrogenase Deficiency
49.
Most common enzyme deficiency of the EMP and is the most common form of hereditary nonspherocytic hemolytic anemia (HNSHA).
a)
Pyruvate Kinase (PK) Deficiency
b)
Glucose-6-phosphate-dehydrogenase Deficiency
50.
Possible PBS findings include: acanthocytes, burr cells
a)
Pyruvate Kinase (PK) Deficiency
b)
Glucose-6-phosphate-dehydrogenase Deficiency
51.
Which of the following is an X-linked recessive disorder?
a)
Pyruvate Kinase (PK) Deficiency
b)
Glucose-6-phosphate-dehydrogenase Deficiency
52.
Most common enzyme deficiency in the pentose phosphate pathway and is also the most common RBC enzyme defect (prevalence of 5% of the global population or approximately 400 million people worldwide)
a)
Pyruvate Kinase (PK) Deficiency
b)
Glucose-6-phosphate-dehydrogenase Deficiency
53.
Possible PBS findings include Heinz bodies and bite cells
a)
Pyruvate Kinase (PK) Deficiency
b)
Glucose-6-phosphate-dehydrogenase Deficiency
54.
Autohemolysis is slightly to moderately increased but is partially corrected by glucose. [TYPE I]
a)
Pyruvate Kinase (PK) Deficiency
b)
Glucose-6-phosphate-dehydrogenase Deficiency
55.
Autohemolysis is greatly increased and glucose has no effect (but ATP corrects the hemolysis) [TYPE II]
a)
Pyruvate Kinase (PK) Deficiency
b)
Glucose-6-phosphate-dehydrogenase Deficiency
56.
Possible PBS findings in G6PD
a)
Acanthocytes
b)
Burr cells
c)
Heinz bodies
d)
Iron
e)
Bite cells
57.
G6PD
a)
Autosomal dominant
b)
Autosomal recessive
c)
X-linked recessive
d)
X-linked dominant
58.
PK deficiency
a)
Autosomal dominant
b)
Autosomal recessive
c)
X-linked recessive
d)
X-linked dominant
59.
Which of the following is the recommended screening test for PK defiicency?
a)
Pk fluorescent spot test
b)
Autohemolysis test
c)
Quantitative PK assay
60.
Which of the following is a screening test for PK deficiency in which there is a greatly increase but glucose has no effect in it?
a)
Pk fluorescent spot test
b)
Autohemolysis test
c)
Quantitative PK assay
61.
Which of the following is a confirmatory test for PK deficiency?
a)
Pk fluorescent spot test
b)
Autohemolysis test
c)
Quantitative PK assay
62.
What are the possible PBS findings in Pyruvate Kinase deficiency?
a)
Stomatocytes
b)
Acanthocytes
c)
Echinocytes
d)
Burr cells
e)
Sickle cells
63.
G6PD Enzyme activity is Severely deficient: <1% activity or not detectable
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
64.
G6PD Enzyme activity is Severely deficient: <10% activity
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
65.
G6PD Enzyme activity is Mild to moderately deficient 10% to 60% activity
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
66.
G6PD Enzyme activity is Mildly deficient to normal: 60% to 150% activity
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
67.
G6PD Enzyme activity is Increased: >150% activity
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
68.
Clinical manifestations include chronic, hereditary nonspherocytic hemolytic anemia (HNSHA), ; severity is variable; rare
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
69.
Clinical manifestations include Severe, episodic acute hemolytic anemia associated with infections, certain drugs, and fava beans; not self-limited and may require transfusions during hemolytic episodes
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
70.
Clinical manifestations include Episodic, acute hemolytic anemia associated with infections and certain drugs; self-limited
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
71.
G6PD-Serres
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
72.
G6PD-Madrid
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
73.
G6PD-Mediterranean
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
74.
G6PD-Chatham
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
75.
G6PD-A
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
76.
G6PD-Canton
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
77.
G6PD-B (wildtype)
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
78.
Which of the following have no clinical manifestation?
a)
G6PD Deficiency Class I
b)
G6PD Deficiency Class II
c)
G6PD Deficiency Class III
d)
G6PD Deficiency Class IV
e)
G6PD Deficiency Class V
79.
Only a small group of G6PD-deficient individuals demonstrate favism, and most of these have the G6PD-_____ variant
a)
G6PD-Serres
b)
G6PD-Madrid
c)
G6PD-Mediterranean
d)
G6PD-Chatham
80.
larger than normal RBCs (diameter > 8.0 um)
a)
Macrocytes
b)
Microcytes
81.
Which of the following is usually seen when the MCV is > 100 fL?
a)
Macrocytes
b)
Microcytes
82.
Which of the following is usually seen when the MCV is < 80 fL?
a)
Macrocytes
b)
Microcytes
83.
Which of the following is associated with defective hemoglobin formation?
a)
Macrocytes
b)
Microcytes
84.
Which of the following is associated with impaired DNA synthesis?
a)
Macrocytes
b)
Microcytes
85.
smaller than normal RBCs (diameter < 6.0 um)
a)
Macrocytes
b)
Microcytes
86.
smaller than normal RBCs (diameter < 6.0 um)
a)
Macrocytes
b)
Microcytes
87.
Which part of the RBC histogram provides a visual display of cell size?
a)
X-axis
b)
Y-axis
88.
automated hematology analyzers produce histograms for all of the following except;
a)
RBCs
b)
WBCs
c)
Platelets
d)
Hematocrit
89.
If the RBCs are macrocytic, the curve will
a)
shift to the right
b)
shift to the left
c)
bimodal
90.
If the RBCs are microcytic, the curve will
a)
shift to the right
b)
shift to the left
c)
bimodal
91.
Happens when there are two populations of RBCs in the sample; example, blood transfusion (when normocytic donor erythrocytes are transfused to a recipient with microcytic red cells), cold agglutinin disease, and hemolytic anemia with schistocytes present
a)
shift to the right
b)
shift to the left
c)
bimodal
92.
a calculated index (from the RBC histogram) given by hematology analyzers to help identify anisocytosis and provide information about its degree
a)
MCV
b)
RDW
c)
MCHC
d)
MCH
93.
Which part of the RBC histogram provides the cell frequency or number of cells?
a)
X-axis
b)
Y-axis
94.
Based on both the width of the RBC distribution curve and the mean RBC size
a)
RDW-CV
b)
RDW-SD
95.
-earliest method provided by the hematology analyzers to measure red cell variations
a)
RDW-CV
b)
RDW-SD
96.
dependent on the width of the distribution curve and the MCV
a)
RDW-CV
b)
RDW-SD
97.
better and more reliable measure of erythrocyte variability, specifically in highly abnormal conditions
a)
RDW-CV
b)
RDW-SD
98.
NOT influenced by the MCV
a)
RDW-CV
b)
RDW-SD
99.
width of the curve is measured at the point that is 20% above the baseline
a)
RDW-CV
b)
RDW-SD
100.
Reference range in adults is 39 to 46 fL
a)
RDW-CV
b)
RDW-SD
101.
Reference range in adults is 11.5% to 14.5 %
a)
RDW-CV
b)
RDW-SD
102.
Based on the actual measurement of the width of the RBC distribution curve in fL (femtoliters)
a)
RDW-CV
b)
RDW-SD
103.
Anemia of chronic inflammation (ACI)
a)
Decreased MCV (microcytic)
b)
Normal MCV (normocytic)
c)
Increased MCV (macrocytic)
104.
It is the variation int he size of red blood cells
a)
Poikilocytosis
b)
Anisochromia
c)
Anisocytosis
d)
Spherocytosis
105.
Iron deficiency anemia
a)
Decreased MCV (microcytic)
b)
Normal MCV (normocytic)
c)
Increased MCV (macrocytic)
106.
Sickle cell anemia
a)
Decreased MCV (microcytic)
b)
Normal MCV (normocytic)
c)
Increased MCV (macrocytic)
107.
G6PD deficiency
a)
Decreased MCV (microcytic)
b)
Normal MCV (normocytic)
c)
Increased MCV (macrocytic)
108.
liver disease
a)
Decreased MCV (microcytic)
b)
Normal MCV (normocytic)
c)
Increased MCV (macrocytic)
109.
megaloblastic anemia
a)
Decreased MCV (microcytic)
b)
Normal MCV (normocytic)
c)
Increased MCV (macrocytic)
110.
ACI
a)
Normal (little or no anisocytosis)
b)
Increased (anisocytosis)
111.
IDA
a)
Normal (little or no anisocytosis)
b)
Increased (anisocytosis)
112.
Sickle cell anemia
a)
Normal (little or no anisocytosis)
b)
Increased (anisocytosis)
113.
Megaloblastic anemia
a)
Normal (little or no anisocytosis)
b)
Increased (anisocytosis)
114.
Liver disease
a)
Normal (little or no anisocytosis)
b)
Increased (anisocytosis)
115.
G6PD deficiency
a)
Normal (little or no anisocytosis)
b)
Increased (anisocytosis)
100 %
