1.

Which of the following findings is

consistent with a diagnosis of
megaloblastic anemia?
SEPTEMBER 6, 2021 a. Hyposegmentation of neutrophils
HEMATOLOGY b. Decreased serum lactate
ASSESSMENT dehydrogenase level
c. Absolute increase in reticulocytes
d. Increased MCV

Characteristic features • Megaloblastic anemias are caused by

•M conditions that impair synthesis of
_____, such as _______________or
•O
_____________.
•B
•P
• _______ maturation lags behind
•H cytoplasmic development as a result
•C of the impaired DNA synthesis.
•D

• This asynchrony between nuclear and • A megaloblastic anemia is

cytoplasmic development results in larger
cells.
• All cells of the body are ultimately affected
by the defective production of DNA
• _________ anemia is one cause of vitamin
B12 deficiency, whereas malabsorption
secondary to ___________________ is
one cause of folate deficiency.
characterized by oval macrocytes
and hypersegmented neutrophils in
the peripheral blood and by
megaloblasts or large nucleated RBC
precursors in the bone marrow.
• The MCV in megaloblastic anemia can
be markedly increased (up to ___ fL),
but modest increases (___-___ fL)
occur as well.
2. A patient has a clinical picture of
megaloblastic anemia. The serum
folate level is decreased, and the
serum vitamin B12 level is 600 pg/mL
(reference interval is 200–900 pg/mL).
What is the expected value for the
methylmalonic acid assay?
a. Increased
b. Decreased
c. Within the reference interval
• One is isomerization of methylmalonyl
coenzyme A (CoA) to succinyl CoA,
which requires vitamin B12 (in the
adenosylcobalamin form) as a cofactor
and is catalyzed by the enzyme
methylmalonyl CoA mutase.
• Folate is the general term used for
any form of the vitamin folic acid.
• Folic acid is the synthetic form in
supplements and fortified food.
• Folates consist of a pteridine ring
attached to para-aminobenzoate
with one or more glutamate
residues.
• ____________________ is an
essential nutrient consisting of a
tetrapyrrole (corrin) ring containing
cobalt that is attached to 5,6-
dimethylbenzimidazolyl
ribonucleotide.
• Vitamin B12 is a ________ in two
biochemical reactions in humans.
• In the _______ of vitamin B12,
the impaired activity of
methylmalonyl CoA mutase
leads to a ____ level of serum
methylmalonic acid (MMA),
which is useful for the
diagnosis of vitamin B12
deficiency
• The function of folate is to transfer carbon
units in the form of methyl groups from
donors to receptors.
• In this capacity, folate plays an important
role in the metabolism of amino acids and
nucleotides.
• Deficiency of the vitamin leads to impaired
cell replication and other metabolic
alterations.
3. Which one of the following statements 4. Which of the following CBC
characterizes the relationships among findings is most suggestive of a
macrocytic anemia, megaloblastic
megaloblastic anemia?
anemia, and pernicious anemia?
a. Macrocytic anemias are megaloblastic. a. MCV of 103 fL
b. Macrocytic anemia is pernicious b. Hypersegmentation of neutrophils
anemia. c. RDW of 16%
c. Megaloblastic anemia is macrocytic. d. Hemoglobin concentration of 9.1
d. Megaloblastic anemia is pernicious g/dL
anemia.

5. In the following description of a bone marrow smear, find

the statement that is inconsistent with the expected picture 6. Which one of the following findings
in megaloblastic anemia. “The marrow appears would be inconsistent with elevated
hypercellular with a myeloid-to-erythroid ratio of 1:1 due to
prominent erythroid hyperplasia. Megakaryocytes appear titers of intrinsic factor blocking
normal in number and appearance. The WBC elements
appear larger than normal, with especially large antibodies?
metamyelocytes, although they otherwise appear
morphologically normal. The RBC precursors also appear a. Hypersegmentation of neutrophils
large. There is nuclear-cytoplasmic asynchrony, with the
nucleus appearing more mature than expected for the color b. Low levels of methylmalonic acid
of the cytoplasm.”
a. Erythroid nuclei that are more mature than cytoplasm
c. Macrocytic RBCs
b. Larger than normal WBC elements d. Low levels of vitamin B12
c. Larger than normal RBCs
d. Normal appearance of megakaryocytes

7. Which of the following is the

• The vitamin ________________ complex,
most metabolically active form of termed ______________________, is the
absorbed vitamin B12? metabolically active form of vitamin B12.
a. Transcobalamin • ______________ binds to specific
receptors on the surfaces of many different
b. Intrinsic factor–vitamin B12 types of cells and enters the cells by
complex endocytosis, with subsequent release of
c. Holotranscobalamin vitamin B12 from the carrier.
• The body maintains a substantial reserve of
d. Haptocorrin–vitamin B12 absorbed vitamin B12 in _________.
complex

• In the plasma, only 10% to
30% of the vitamin B12 is
bound to _____________; the
remaining 75% is bound to
transcobalamin I and III,
referred to as the _________.
• Vitamin B12 and folate are needed for
the production of _______
___________ for DNA synthesis.
• Deficiencies of either vitamin impair
DNA replication, halt cell division, and
increase apoptosis, which results in
ineffective hematopoiesis and
megaloblastic morphology of
erythrocyte precursors.
• Folate deficiency may result from
inadequate intake, increased need with
growth or pregnancy, impaired absorption,
impaired use, or excessive loss.
• The action of folate can be impaired by
drugs such as those used to treat epilepsy
or cancer.
• Renal dialysis patients experience
significant folate loss to the dialysate.
8. Folate and vitamin B12
work together in the
production of:
a. Amino acids
b. RNA
c. Phospholipids
d. DNA
• Lack of vitamin B12 leads to the
accumulation of
___________________________
___________________________.
• Folate deficiency, in particular,
leads to elevation of _________
levels and possible risk of
_____________________.
9. The macrocytosis associated with megaloblastic
anemia results from:
a. Reduced numbers of cell divisions with normal
cytoplasmic development
b. Activation of a gene that is typically active only
in megakaryocytes
c. Reduced concentration of hemoglobin in the
cells so that larger cells are needed to provide the
same oxygen-carrying capacity
d. Increased production of reticulocytes in an
attempt to compensate for the anemia
• ________________ often is the
earliest sign of megaloblastic
anemia.
• Patients with uncomplicated
megaloblastic anemia are
expected to have _________
hemoglobin and hematocrit
values, __________, and
_____________.
• Pernicious anemia is an
___________ disorder
characterized by impaired
absorption of vitamin B12 due to a
lack of intrinsic factor.
• This condition is called pernicious
anemia because the disease was
___________________________
______.
11. The clinical consequences of
pancytopenia include:
a. Pallor and thrombosis
b. Kidney failure and fever
c. Fatigue, infection, and bleeding
d. Weakness, hemolysis, and
infection
10. Which one of the following
groups has the highest risk for
pernicious anemia?
a. Malnourished infants
b. Children during growth periods
c. Persons older than 60 years of
age
d. Pregnant women
• The incidence per year is
roughly __ new cases per
100,000 persons older than 40
years of age.
• Pernicious anemia most often
manifests in the ____ decade
or later.
12. Idiopathic acquired aplastic
anemia is due to a(n):
a. Drug reaction
b. Benzene exposure
c. Inherited mutation in stem
cells
d. Unknown cause
 Etiologic Classification of Aplastic Anemia
Acquired (__% to __% of cases)
*Idiopathic (__% of cases)
*Secondary (__% to __% of cases)
-Dose dependent/predictable
• Cytotoxic drugs
*Inherited (__% to __% of
• Benzene
• Radiation
- Idiosyncratic
cases)
•F
•Drugs
•Chemicals
Insecticides
Cutting/lubricating oils
-Viruses
• Epstein-Barr virus
•D
• Hepatitis virus (non-A, non-B, non-C, non-G)
• Human immunodeficiency virus
- Miscellaneous conditions
•S
• Paroxysmal nocturnal hemoglobinuria
• Autoimmune diseases
• Pregnancy

13. The pathophysiologic mechanism in • Bone marrow failure in

acquired idiosyncratic aplastic anemia is:
• a. Replacement of bone marrow by
acquired aplastic anemia
abnormal cells occurs from destruction of
• b. Destruction of stem cells by autoimmune hematopoietic stem cells by
T cells direct toxic effects of a drug,
• c. Defective production of hematopoietic
growth factors
autoimmune T-cell targeting of
• d. Inability of bone marrow stroma to stem cells, or other unknown
support stem cells mechanisms.

14. The most consistent peripheral blood

• The autoimmune reactions are findings in severe aplastic anemia are:
rare adverse events after a. Hairy cells, monocytopenia, and
exposure to drugs, chemicals, or neutropenia
viruses. b. Macrocytosis, thrombocytopenia, and
neutropenia
• They are idiosyncratic in that they
c. Blasts, immature granulocytes, and
are unpredictable, and severity is thrombocytopenia
unrelated to the dose or duration d. Polychromasia, nucleated RBCs, and
of exposure. hypersegmented neutrophils
15. The treatment that has shown the best
success rate in young patients with severe
aplastic anemia is:
a. Immunosuppressive therapy
b. Long-term red blood cell and platelet
transfusions
c. Administration of hematopoietic growth
factors and androgens
d. Stem cell transplant with an HLA-identical
sibling
• Preferred treatment for severe and
very severe acquired aplastic anemia
is hematopoietic stem cell
transplant (HSCT) for younger
patients with an HLA-identical
sibling.
• For those without a matched sibling
donor and for those who are not HSCT
candidates, immunosuppressive
therapy with ____________ ______
and _________ is recommended.
• Fanconi anemia (FA), dyskeratosis
congenita (DKC), and Shwachman-
Bodian-Diamond syndrome (SBDS) are
inherited forms of aplastic anemia with
progressive ________________, and
patients may present with characteristic
________________.
• FA is inherited in an
_____________________________
pattern, and mutations in __ genes have
been identified.
• Aplastic anemia is classified as
nonsevere, severe, or very severe,
based on bone marrow
___________, absolute ________
count, ______ count, ________
level, and _________ count.
• The severity classification helps to
guide treatment decisions.
16. The test that is most useful in
differentiating FA from other causes of
pancytopenia is:
a. Bone marrow biopsy
b. Ham acidified serum test
c. Diepoxybutane-induced
chromosome breakage
d. Flow cytometric analysis of CD55
and CD59 cells
• A positive chromosome breakage study
with diepoxybutane is diagnostic.
• DKC can be
________________________________
________________________________,
and mutations in __ genes have been
identified.
• SBDS is ______________ and is
associated with mutations in the SBDS
gene.
17. Mutations in genes that code for the
telomerase complex may induce bone marrow
failure by causing which one of the following?
a. Resistance of stem cells to normal apoptosis
b. Autoimmune reaction against telomeres in
stem cells
c. Decreased production of hematopoietic
growth factors
d. Premature death of hematopoietic stem cells
18. Diamond-Blackfan anemia
differs from inherited aplastic
anemia in that in the former:
a. Reticulocyte count is increased
b. Fetal hemoglobin is decreased
c. Only erythropoiesis is affected
d. Congenital malformations are
absent
19. Which anemia should be suspected
in a patient with refractory anemia,
reticulocytopenia, hemosiderosis, and
binucleated erythrocyte precursors in
the bone marrow?
a. Fanconi anemia
b. Dyskeratosis congenita
c. Acquired aplastic anemia
d. Congenital dyserythropoietic anemia
• Telomerase complex defects play a
role in the pathophysiology of inherited
aplastic anemias and some acquired
aplastic anemias.
• The defects result in the
______________________________
______________________________
which leads to premature
hematopoietic stem cell _________
and _________.
• Pure red cell aplasia is a disorder of
__________ production.
• Acquired (TEC and DBA)
___________________________________
___________________________________
________________________________are
disparate subtypes with distinct etiologies,
clinical features, and courses.
• Mutations in __ different ribosomal protein
genes have been identified in DBA.
• The congenital dyserythropoietic
anemias (CDAs) are a heterogeneous
group of rare disorders characterized
by refractory anemia,
reticulocytopenia, hypercellular bone
marrow with markedly ineffective
erythropoiesis, and distinctive
dysplastic changes in bone marrow
.
erythroblasts.

• Secondary hemosiderosis arises
from chronic ____________ and
___________ hemolysis as well
as increased iron absorption
associated with ineffective
erythropoiesis.
• Iron overload develops even in the
absence of blood transfusions.
_________
• A type low-risk MDS anemia
characterized by mono-lineage
dysplasia, anemia,
dyserythropoiesis, and low
percentage of blasts in bone
marrow and peripheral blood.

_____________ ____________
• Physical malformation • Hypocellular; blasts and abnormal cells
• Macrocytosis absent;
• Elevated HbF
• Anemia • Reticulin normal; RBC dyspoiesis may be
• Neutropenia present;
• Thrombocytopenia • WBC and platelet dyspoiesis absent
• Duplications and triplications of the long arm of
• PNH cells* may be present;
_______________.
• Gains of the portions of long arms of __________ • Chromosome abnormalities may be
• Monosomy __ present
• No splenomegaly

_____________ 20. The primary pathophysiologic

mechanism of anemia associated with
• Moderate Pancytopenia chronic kidney disease is:
• Marked hypocellular marrow a. Inadequate production of
(<10%) erythropoietin
b. Excessive hemolysis
• Reduced trilineage
c. Hematopoietic stem cell mutation
hematopoiesis
d. Toxic destruction of stem cells
• No evidence of malignancy
• Anemia is a common complication of • Without erythropoietin, the
chronic kidney disease (CKD), with a bone marrow lacks adequate
positive correlation between anemia stimulation to produce RBCs.
and renal disease severity
• Another contributor to the
• The primary cause of anemia in CKD
is ___________________________. anemia of CKD is _____, which
inhibits erythropoiesis and
increases RBC fragility.

21. The term hemolytic disorder in general

refers to a disorder in which there is:
a. Increased destruction of RBCs after they
enter the bloodstream
b. Excessive loss of RBCs from the body
c. Inadequate RBC production by the bone
marrow
d. Increased plasma volume with unchanged
red cell mass

Differential Diagnosis for hemolytic

22. RBC destruction that occurs anemias
when macrophages ingest and
destroy RBCs is termed:
a. Extracellular
b. Macrophage-mediated
c. Intra-organ
d. Extrahematopoietic
23. A sign of hemolysis that is
typically associated with both
fragmentation and macrophage-
mediated hemolysis is:
a. Hemoglobinuria
b. Hemosiderinuria
c. Hemoglobinemia
d. Elevated urinary urobilinogen level
• When only the
____________________ fraction
of the total serum bilirubin is
elevated, hemolytic jaundice is
confirmed.
• An elevated urinary ___________
level strengthens the conclusion.
•RBC morphology and
haptoglobin levels can
assist in differentiating
fragmentation from a
macrophage-mediated
cause.
• A rapid decrease in hemoglobin
concentration (e.g., 1 g/dL per week)
from levels previously within the
reference interval can signal
hemolysis when _________ and
_________ have been ruled out.
• Jaundice and reticulocytosis provide
additional confirmation of a hemolytic
cause for an anemia of at least several
days duration.
• The rapid decrease in hemoglobin
during an acute hemolytic episode,
however, usually is apparent before
reticulocytosis and bilirubinemia
develop.
• For acute hemolysis, hemoglobinemia
and hemoglobinuria are expected with
fragmentation causes; therefore, their
absence suggests a
_______________ cause.
24. An elderly white woman is evaluated for worsening
anemia, with a decrease of approximately 0.5 mg/dL of
hemoglobin each week. The patient is pale, and her
skin and eyes are slightly yellow. She complains of
extreme fatigue and is unable to complete the tasks of
daily living without napping in midmorning and
midafternoon. She also tires with exertion, finding it
difficult to climb even five stairs. Which of the features
of this description points to a hemolytic cause for her
anemia?
a. Pallor
b. Yellow skin and eyes
c. Need for naps
d. Tiredness on exertion
 Laboratory Evaluation of Hemolysis
25. Which of the following tests *Hematologic Intravascular Extravascular
provides a good indication of Blood film
accelerated erythropoiesis? Reticulocyte
a. Urine urobilinogen level Bone marrow
b. Hemosiderin level *Serum or
Plasma
c. Reticulocyte count Bilirubin
d. Glycated hemoglobin level Haptoglobin
Plasma free
hemoglobin

Laboratory Evaluation of Hemolysis

26. A 5-year-old girl was seen by her physician
*Serum or Intravascular Extravascular
several days prior to this visit and was diagnosed
Plasma with pneumonia. Her mother has brought her to the
physician again because the girl’s urine began to
LDH darken after the first visit and now is alarmingly dark.
The girl has no history of anemia, and there is no
*Urine
family history of any hematologic disorder. The CBC
Bilirubin shows a mild anemia, polychromasia, and a few
schistocytes. This anemia could be categorized as:
Hemosiderin
a. Acquired, fragmentation
Hemoglobin b. Acquired, macrophage-mediated
c. Hereditary, fragmentation
Urobilinogen d. Hereditary, macrophage-mediated

27. A patient has a personal and family history of a mild
hemolytic anemia. The patient has consistently elevated
levels of total and indirect serum bilirubin and urinary
urobilinogen. The serum haptoglobin level is consistently
decreased, whereas the reticulocyte count is elevated. The
latter can be seen as polychromasia on the patient’s blood
film, along with spherocytes. Which of the findings reported
for this patient is inconsistent with a classical diagnosis of
fragmentation hemolysis?
• a. Elevated total and indirect serum bilirubin
• b. Elevated urinary urobilinogen
• c. Decreased haptoglobin
• d. Spherocytes on the peripheral film
28. Select the statement that is true about
bilirubin metabolism.
a. Indirect bilirubin is formed in the liver by the
addition of two sugar molecules to direct
bilirubin.
b. Macrophages of the spleen liberate bilirubin
during hemoglobin catabolism.
c. Urobilinogen is not water soluble and is not
excreted in the urine.
d. Normally, the major fraction of bilirubin in the
blood is the direct (conjugated) form released
from macrophages.
29. A patient has anemia that has been worsening over the last
several months. The hemoglobin level has been declining slowly,
30. Which of the following sets of test results
with a drop of 1.5 g/dL of hemoglobin over about 6 weeks. is typically expected with chronic
Polychromasia and anisocytosis are seen on the blood film,
consistent with the elevated reticulocyte count and RBC fragmentation hemolysis?
distribution width (RDW). Serum levels of total bilirubin and
indirect fractions are normal. Urinary urobilinogen level also is • Serum Haptoglobin
normal. When these findings are evaluated, the conclusion is
drawn that the anemia does not have a hemolytic component. • Urine Hemoglobin
Based on the data given here, why was hemolysis ruled out as
the cause of the anemia? • Urine Sediment Prussian Blue Stain
a. The decline in hemoglobin is too gradual to be associated with a. Increased Positive Positive
hemolysis.
b. The elevation of the reticulocyte count suggests a malignant b. Decreased Negative Negative
cause.
c. Evidence of increased protoporphyrin catabolism is lacking. c. Decreased Positive Positive
d. Elevated RDW points to an anemia of decreased production. d. Increased Positive Negative

31. In HS a characteristic • Hereditary spherocytosis arises

abnormality in the CBC results is: from defects in proteins that
• a. Increased MCV provide _______ support for the
• b. Increased MCHC membrane.
• c. Decreased MCH • Hereditary elliptocytosis is due to
• d. Decreased platelet and WBC
defects in cytoskeletal proteins
counts that provide ________ support for
the membrane.

• Diagnostic of Hereditary spherocytosis:

•Blood smear shows _________
Spherocytes and • Blood is added to serial dilutions of NaCl &
polychromosia incubated.
• Amount of hemolysis determined by reading
•Hemoglobin electrophoresis is absorbance of supernatant from each tube.
______. • ↑ in ____________________.
• ↓ with TIST:
•MCHC usually >__ g/dL. ___________________________________
___________________________________.
•___ retics, ___ osmotic fragility.
32. The altered shape of the
spherocyte in HS is due to:
a. An abnormal RBC membrane protein
affecting vertical protein interactions
b. Defective RNA synthesis
c. An extrinsic factor in the plasma
d. Abnormality in the globin
composition of the hemoglobin
molecule
33. Which of the following results are
consistent with HS?
a. Increased osmotic fragility, negative DAT
result
b. Decreased osmotic fragility, positive DAT
result
c. Increased osmotic fragility, positive DAT
result
d. Decreased osmotic fragility, negative DAT
result

34. The RBCs in HE are abnormally shaped
and have unstable cell membranes as a
result of:
a. Abnormal shear stresses in the circulation
b. Defects in horizontal membrane protein
interactions
c. Mutations in ankyrin
d. Lack of all Rh antigens in the RBC
membrane
35. The peripheral blood film for patients
with mild HE is characterized by:
a. Elliptical RBCs
b. Oval RBCs with one or two transverse
ridges
c. Overhydrated RBCs with oval central
pallor
d. Densely stained RBCs with a few
irregular projections

__________- ___________

•Defect in band 3 causing • Severe defect in spectrin that disrupts

horizontal linkages in protein
increased membrane rigidity. cytoskeleton;
•Only exists in heterozygous • Severe RBC fragmentation
State 30% oval cells with • Elliptocytes,

one or two transverse • Schistocytes,

• Microspherocytes
ridges.
 ____________
• Increased membrane permeability to
sodium and potassium;
• increased intracellular sodium causing
influx of water, increase in cell volume,
and decreased cytoplasmic viscosity
• Stomatocytes (5%–50%),
• macrocytes
____________
• Increased membrane permeability
to potassium;
• decreased intracellular potassium,
resulting in loss of water from cell,
• decrease in cell volume,
• increased cytoplasmic viscosity

36. Laboratory test results for patients with 37. Acanthocytes are found in
HPP include all of the following except:
a. RBCs that show marked thermal
association with:
sensitivity at 41° C to 45° C a. Abetalipoproteinemia
b. Marked poikilocytosis with elliptocytes,
RBC fragments, and microspherocytes
b. G6PD deficiency
c. Low fluorescence when incubated with c. Rh deficiency syndrome
eosin-5’-maleimide
d. Increased MCV and normal RDW
d. Vitamin B12 deficiency

Pappenheimer
38. The most common manifestation of
G6PD deficiency is:
a. Chronic hemolytic anemia caused by cell
shape change
b. Acute hemolytic anemia caused by drug
exposure or infections
c. Mild compensated hemolysis caused by
ATP deficiency
d. Chronic hemolytic anemia caused by
intravascular RBC lysis
• Oxidation converts hemoglobin to
___________ and forms sulfhydryl
groups and disulfide bridges in
hemoglobin polypeptides.
• This leads to decreased
hemoglobin solubility and
precipitation as _____ bodies
• Reticulocytosis typically occurs as a
response to an acute hemolytic episode
and will falsely increase the patient’s G6PD
activity over baseline values.
• Patients who are not G6PD deficient are
expected to have high G6PD activity during
hemolytic episodes.
• When a patient with reticulocytosis has
an unexpectedly normal G6PD activity
result, G6PD deficiency should be
suspected.
• G6PD-deficient RBCs cannot generate
sufficient ______ to reduce ________
and thus cannot effectively detoxify
the hydrogen peroxide produced upon
exposure to oxidative stress.
• Oxidative damage to cellular proteins
and lipids occurs, particularly affecting
hemoglobin and the cell membrane.
39. A patient experiences an episode of
acute intravascular hemolysis after taking
primaquine for the first time. The physician
suspects that the patient may have G6PD
deficiency and orders an RBC G6PD assay
2 days after the hemolytic episode begins.
How will this affect the test result?
a. No effect
b. False increase due to reticulocytosis
c. False decrease due to hemoglobinemia
d. Absence of enzyme activity
• To avoid falsely elevated or falsely
normal results, biochemical assays for
G6PD activity should not be performed
during _____________.
• The testing should be performed after
the reticulocyte and total RBC counts
have returned to baseline, which may
take __ weeks to __ months after the
hemolytic episode
40. The most common defect or
deficiency in the anaerobic glycolytic
pathway that causes chronic HNSHA
is:
a. Pyruvate kinase deficiency.
b. Lactate dehydrogenase deficiency
c. Glucose-6-phosphate
dehydrogenase deficiency
d. Methemoglobin reductase deficiency
• Patients with classes II and III G6PD
variants may develop acute hemolytic
anemia after infections or after
ingestion of certain ______ or ______
• A small percentage of patients have
class I G6PD variant and chronic
hereditary nonspherocytic hemolytic
anemia (HNSHA).
41. Which of the following laboratory
tests would be best to confirm PNH?
a. Acidified serum test (Ham test)
b. Osmotic fragility test
c. Flow cytometry for detection of eosin-
59-maleimide binding on erythrocytes
d. Flow cytometry for detection of CD55,
CD59, and FLAER binding on
neutrophils and monocytes
•_______________________
______________________
is the most common RBC
enzymopathy, but the vast
majority of patients are
asymptomatic.
• Most patients with
_______________ deficiency
have symptoms of hemolysis.
• ____ cells are commonly observed
on the peripheral blood film.
• PK deficiency is the most common
cause of HNSHA.
42. Which one of the following is a
feature found in all microangiopathic
hemolytic anemias?
a. Pancytopenia
b. Thrombocytosis
c. Intravascular RBC fragmentation
d. Prolonged prothrombin time and
partial thromboplastin time
•Microangiopathic hemolytic
anemias (MAHAs) are a
group of potentially life-
threatening disorders
characterized by RBC
_______________________
______________________.
• Upon shearing, RBC membranes
quickly reseal with minimal escape of
hemoglobin, but the resulting
fragments (called schistocytes) are
distorted and become rigid.
• The spleen clears the rigid RBC
fragments from the circulation through
the extravascular hemolytic process
• The major microangiopathic
hemolytic anemias include
______________________ (TTP),
____________________ (HUS),
HELLP
(___________________________
__________________________)
syndrome, and ____.
• The RBC fragmentation occurs
intravascularly by the mechanical
shearing of RBC membranes as
the cells rapidly pass through
turbulent areas of small blood
vessels that are partially blocked
by microthrombi or damaged
endothelium
43. Typical laboratory findings in TTP
and HUS include:
a. Schistocytosis and thrombocytopenia
b. Anemia and reticulocytopenia
c. Reduced levels of lactate
dehydrogenase and aspartate
aminotransferase
d. Increased levels of free plasma
hemoglobin and serum haptoglobin
44. The pathophysiology of idiopathic TTP
involves:
a. Shiga toxin damage to endothelial cells and
obstruction of small blood vessels in glomeruli
b. Formation of platelet-VWF thrombi due to
autoantibody inhibition of ADAMTS-13
c. Overactivation of the complement system and
endothelial cell damage due to loss of regulatory
function
d. Activation of the coagulation and fibrinolytic
systems with fibrin clots throughout the
microvasculature
• TTP can be idiopathic, secondary, or
inherited.
• Idiopathic TTP has no known
precipitating event.
• In idiopathic TTP, autoantibodies to
___________ inhibit its activity,
causing a severe deficiency.
• These autoantibodies are usually of
the ___ class but can be ___ or ___.
• Secondary TTP is very
heterogeneous, and the
mechanisms that trigger the
TTP pathophysiology are not
completely clear.
46. Which of the following species of
Plasmodium produce hypnozoites that
can remain dormant in the liver and
cause a relapse months or years later?
a. P. falciparum
b. P. vivax
c. P. knowlesi
d. P. malariae
• Secondary TTP can be triggered by infections,
pregnancy, surgery, trauma, inflammation, and
disseminated malignancy, possibly by depressing
the synthesis of ADAMTS-13.
• Other conditions may induce an inhibitory
reaction to ADAMTS-13, including (HAHQTT)
_______________________________________
_______________________________________
_______________________________________
_______________________________________.
45. Which one of the following laboratory
results may be seen in BOTH traumatic
cardiac hemolytic anemia and exercise-
induced hemoglobinuria?
a. Schistocytes on the peripheral blood
film
b. Thrombocytopenia
c. Decreased serum haptoglobin
d. Hemosiderinuria
47. Which one of the following is not a
mechanism causing anemia in P. falciparum
infections?
a. Inhibition of erythropoiesis
b. Lysis of infected RBCs during schizogony
c. Competition for vitamin B12 in the
erythrocyte
d. Immune destruction of noninfected RBCs
in the spleen
48. Which Plasmodium species is
widespread in Malaysia, has RBCs with
multiple ring forms, has band-shaped early
trophozoites, shows a 24-hour erythrocytic
cycle, and can cause severe disease and
high parasitemia?
• a. P. falciparum
• b. P. vivax
• c. P. knowlesi
• d. P. malariae
50. What RBC morphology is
characteristically found within the first
24 hours following extensive burn
injury?
a. Macrocytosis and polychromasia
b. Burr cells and crenated cells
c. Howell-Jolly bodies and bite cells
d. Schistocytes and microspherocytes
52. Immune hemolytic anemia is due to
a(n):
a. Structural defect in the RBC
membrane
b. Allo- or autoantibody against an RBC
antigen
c. T cell immune response against an
RBC antigen
d. Obstruction of blood flow by
intravascular thrombi
49. One week after returning from a vacation in Rhode
Island, a 60-year-old man experienced fever, chills,
nausea, muscle aches, and fatigue of 2 days’ duration. A
complete blood count (CBC) showed a WBC count of 4.5 x
109/L, hemoglobin level of 10.5 g/dL, a platelet count of
134 x 109/L, and a reticulocyte count of 2.7%. The medical
laboratory scientist noticed tiny ameboid ring forms in some
of the RBCs and some tetrad forms in others. These
findings suggest:
a. Bartonellosis
b. Malaria
c. Babesiosis
d. Clostridial sepsis
51. Which of the following tests yields
results that are abnormal in DIC but are
usually within the reference interval or just
slightly abnormal in TTP and HUS?
a. Indirect serum bilirubin and serum
haptoglobin
b. Prothrombin time and partial
thromboplastin time
c. Lactate dehydrogenase and aspartate
aminotransferase
d. Serum creatinine and serum total protein
53. The pathophysiology of immune
hemolysis with IgM antibodies
always involves:
• a. Complement
• b. Autoantibodies
• c. Abnormal hemoglobin
molecules
• d. Alloantibodies
54. In hemolysis mediated by IgG
antibodies, which abnormal RBC
morphology is typically observed
on the peripheral blood film?
a. Spherocytes
b. Nucleated RBCs
c. RBC agglutination
d. Macrocytes
• IgG antibodies are not efficient in
activating complement, and
intravascular hemolysis by full
activation of complement from C1
to C9 is rare (except with _____ in
paroxysmal cold hemoglobinuria)
55. The most important finding in the
diagnostic investigation of a suspected
autoimmune hemolytic anemia is:
a. Detection of a low hemoglobin and
hematocrit
b. Observation of hemoglobinemia in a
specimen
c. Recognition of a low reticulocyte count
d. Demonstration of IgG and/or C3d on the
RBC surface
• Hemolysis mediated by IgG antibodies
occurs with or without complement
and predominantly by extravascular
mechanisms.
• RBCs sensitized with IgG are removed
from circulation by macrophages in the
spleen, which have receptors for the
Fc component of _____ and ____.
• Often, IgG-sensitized RBCs are
only partially phagocytized by
macrophages, which results in the
removal of some membrane.
• ________ are the result of this
process, and they are the
characteristic cell of IgG-mediated
hemolysis
56. A qualitative abnormality in hemoglobin
may involve all of the following except:
a. Replacement of one or more amino acids
in a globin chain
b. Addition of one or more amino acids in a
globin chain
c. Deletion of one or more amino acids in a
globin chain
d. Decreased production of a globin chain

• The types of genetic mutations
that occur in the
hemoglobinopathies include
point mutations, deletions,
insertions, and fusions
involving one or more of the
adult globin genes
(___________)
57. The substitution of valine for
glutamic acid at position 6 of the B
chain of hemoglobin results in
hemoglobin that:
a. Is unstable and precipitates as Heinz
bodies
b. Polymerizes to form tactoid crystals
c. Crystallizes in a hexagonal shape
d. Contains iron in the ferric (Fe31)
state
• Deletions involve the removal of one or
more nucleotides, whereas insertions result
in the addition of one or more nucleotides.
• Usually deletions and insertions are not
divisible by three and disrupt the reading
frame, which leads to the nullification of
synthesis of the corresponding globin
chain.
• This is the case for the quantitative
__________.
58. Patients with SCD usually do not
exhibit symptoms until 6 months of age
because:
a. The mother’s blood has a protective
effect
b. Hemoglobin levels are higher in
infants at birth
c. Higher levels of Hb F are present
d. The immune system is not fully
developed
• Individuals affected with SCD are
characteristically symptom free until the
second half of the first year of life because • Erythrocytes containing Hb S
of the protective effect of Hb F. become susceptible to
hemolysis, and a progressive
• Toward the end of the first 6 months of life,
mutated __ chains begin to be produced
hemolytic anemia and
and gradually replace normal __ chains, splenomegaly may become
which causes Hb S levels to ______ and evident.
Hb F levels to _______.

59. Which of the following is the most

definitive test for Hb S?
• a. Hemoglobin solubility test
• b. Hemoglobin electrophoresis at
alkaline pH
• c. Osmotic fragility test
• d. Hemoglobin electrophoresis at acid
pH

60. Which of the following is the principle for

WBC measurement in Abbott CELL-DYN
Sapphire?
a. Impedance volume and conductivity and
five-angle light scatter measurement
b. Fluorescent staining; forward light scatter
and side fluorescent light detection
c. Light scatter and impedance
d. Light scatter and absorption