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Hematology Questions

1. All of the following are characteristics of pluripotent hematopoietic stem cells, except
A. morphologically recognizable
C. express the stem cell marker CD13
B. produce lineage-committed progenitors D. posses self-renewal ability
Hematopoietic stem cells can make copies of themselves to maintain the stem cell pool and posses the
ability to generate cells of all lineages. These stem cells give rise to multipotential myeloid and
lymphoid progenitor cells, which ultimately produce progenitor cells that are restricted to a specific
lineage. With appropriate cytokine stimulus, the committed progenitor cells undergo proliferation to
recognizable precursors that produce an amplified number of mature end-stage cells. Stem cells and
progenitor cells cannot be morphologically distinguished but can be identified phenotypically by
markers such as the stem cell marker CD34. CD34 expression is lost as antigens for a specific cell
lineage are expressed. CD13 is a marker expressed by myeloid precursors.
2. What is the normal ratio of myeloid to erythroid precursors in bone marrow?
A. 8:1
B. 4:1
C. 2:1
D. 1:1
The myeloid to erythroid ratio is the ratio between all granulocytes and their precursors and all
nucleated red cell precursors. Myeloid precursors outnumber erythroid precursors by about 3 or 4 to 1
in the normal bone marrow. Although there are many more red blood cells in the peripheral blood than
granulocytes, red blood cells have much longer life span in circulation (120 days) as compared to
granulocytes (about 8 hours). Granulocytes therefore, require a more continual production than
erythrocytes and are the most numerous marrow precursors. Alterations in the M:E ratio, such as 1:1 or
8:1, may indicate erythroid hyperplasia or granulocytic hyperplasia, respectively.
3. Antigen-independent lymphopoiesis occurs in which of the following sites?
A. thymus and bone marrow
C. spleen and lymph nodes
B. liver and kidney
D. Peyers patches and spleen
Antigen-independent lymphopoiesis occurs in primary lymphoid tissues located in the thymus and bone
marrow. The formation of immunocompetent T and B cells from precursor cells is influenced by the
environment (thymus and bone marrow) and several interleukins. Antigen-dependent lymphopoiesis
occurs in secondary lymphoid tissues (spleen, lymph nodes, Peyers patches of gastrointestinal tract)
and begins with antigenic stimulation of immunocompetent cells.
4. What is the largest hematopoietic cell in normal bone marrow?
A. megakaryoblast
B. osteoclast
C. megakaryocyte
D. plasma cell
The largest hematopoietic cell in normal bone marrow is the mature megakaryocyte. It has a multilobed
nucleus and abundant granular cytoplasm. Plasma cells are characterized by a round, eccentric
nucleus and intensely blue cytoplasm. Osteoblasts and osteoclasts are non hematopoietic cells that
may be present in normal bone marrow.
5. Which erythrocyte precursor has the following characteristics: 12 to 17 mm diameter, N:C of 4:1,
nucleoli not usually apparent, and basophilic cytoplasm?
A. rubriblast
B. rubricyte
C. metarubricyte
D. prorubricyte
The second stage, the prorubricyte or basophilic normoblast, has an overall cell diameter of 12 to 17
mm and is only slightly smaller than the rubriblast. The N:C ratio remains high (4:1); however, this
stage demonstrates morphological evidence of increasing maturity. The cytoplasm continues to appear
basophilic with a Wright stain.
6. What is the primary site of erythropoietin production in adult?
A. kidney
B. liver
C. bone marrow
D. spleen
The substance erythropoietin is produced primarily by the kidneys. Peritubular cells are the probable
site of synthesis in the kidneys. Ten to fifteen percent of erythropoietin production occurs in the liver,
which is the primary source of erythropoietin in the unborn. This glycoprotein hormone, with a molecular
weight of 46,000, stimulates erythropoiesis and can cross the placental barrier between the mother and
the fetus.
7. During which stage of erythrocytic development does hemoglobin production begin?
A. reticulocyte
B. metarubricyte
C. rubricyte
D. prorubricyte
In normal erythrocytic maturation, hemoglobin formation in the late polychromatic normoblast stage
gives the cytoplasm a prominent pink color. The red cell continues to produce hemoglobin throughout
the reticulocyte stage of development.
8. The Embden-Meyerhof glycolytic pathway uses how many percent of the erythrocytes total glucose?
A. 5
B. 10
C. 50
D. 90
The mature red cell lacks mitochondria and, therefore, oxidative phosphorylation and Krebs cycle
activity. Energy production is mainly glycolytic, 90% of which occurs through this pathway. Glucose is
metabolized to lactic acid with the net production of 2 moles of ATP.
9. The function of the methemoglobin reductase pathway is to
A. prevent the oxidation of heme iron
C. maintain iron in ferric state
B. provide cellular energy
D. control the rate of glycolysis
Methemoglobin reductase pathway maintains the iron present in the hemoglobin molecule in its
reduced state, Fe2+ through the action of the enzyme methemoglobin cytochrome C reductase.
10. What is the major type of leukocyte seen in the peripheral smear of a patient with aplastic anemia?
A. neutophil
B. lymphocyte
C. monocyte
D. eosinophil

Lymphocytes constitute the majority of the nucleated cells seen. The bone marrow in aplastic anemia is
spotty with patches of normal cellularity. Lymphocyte production is least affected.
11. Rouleaux of red blod cells when seen in a peripheral blood smear is characteristic of?
A. hypersplenism
C. cold hemagglutinin disease
B. hypogammaglobulinemia
D. multiple myeloma
The presence of red cell rouleaux (coining pattern) is a characteristic finding in multiple myeloma
because of the increased concentration of immunoglobulins in the blood plasma
(hypergammaglobulinemia). The excessive immunoglobulins are produced by malignant plasma cells.
Cold hemagglutinin disease is characterized by cell agglutination or clumping in a nonspecific pattern.
"Hypersplenism" refers to an enlarged, overactive spleen that destroys both normal and abnormal cells,
possibly causing pancytopenia.
12. The presence of schistocytes in a peripheral blood smear is commonly associated with
A. increased iron mobilization
C. decreased erythropoietin activity
B. increased red cell destruction
D. decreased red cell proliferation
The presence of schistocytes (schizocytes) indicates that red cells have been subjected to some form
of physical trauma that causes damage. Red cell fragmentation can be the result of impact with fibrin
strands, mechanical trauma by artificial surfaces, injury by heat, partial phagocytosis, or damage by
toxins and drugs. Schistocytes are characteristic of the increased red blood cell destruction that occurs
in severe hemolytic anemias but are not associated with anemias that result from defective bone
marrow delivery of red cells to the blood.
13. Abetalipoproteinemia is characterized by mild anemia and the finding of numerous __________ in the
peripheral blood smear.
A. acanthocytes
B. echinocytes
C. elliptocytes
D. stomatocytes
Abetalipoproteinemia, or hereditary acanthocytosis, is a rare autosomal recessive disorder of lipid
metabolism. An absence of serum beta lipoproteinemia, a transport protein, causes abnormal plasma
lipids. The numerous acanthocytes (spur cells) are the result of an alteration in the lipid content of the
red cell membrane. The anemia is mild, but this disorder is associated with progressive neurologic
disease.
14. What is the most common cause of iron deficiency anemia?
A. bleeding
B. inadequate diet
C. gastrectomy
D. intestinal malabsorption
The majority of body iron is found in the hemoglobin of circulating erythrocytes. This means that any
form of bleeding will lead to excessive iron loss. Iron balance is normally very tightly controlled through
absorption rather than excretion. Iron deficiency in males is rare but, if present, it is usually the result of
chronic gastrointestinal bleeding (ulcers, cancer).
15. Hereditary stomatocytosis is characterized by
A. decreased oxygen affinity
C. inefficiency of hemoglobin reduction
B. membrane cation permeability
D. cytoskeletal membrane defect
The major defect in heredity stomatocytosis is altered permeability of the red cell membrane to Na+
and K+ ions. A net gain of Na+ within the cell leads to inc. H2O entry and the appearance of swollen cell
with a slit like area of pallor.
16. The following are causes of absolute secondary erythrocytosis, except
A. defective cardiac function
C. dehydration following intake of diuretics
B. high altitude adjustment
D. hemoglobin with increased oxygen affinity
Dehydration is a cause of relative (pseudo) erythrocytosis due to plasma loss while defective
cardiac/pulmonary function, high attitude adjustment and defective oxygen transport are all causes of
absolute secondary erythrocytosis.
17. The following are true of congenital dyserythropoieticanemias (CDAs) except
A. multinucleated erythroblasts
C. elevated M:E ratio
B. ineffective erythropoiesis
D. increased unconjugated bilirubin
There are four classifications of CDAs, each characterized by ineffective erythropoiesis, increased
unconjugated bilirubin, and bizarre multinucleated erythroid precursors.
18. Which of the following conditions may produce spherocytes in a peripheral smear?
A. Pelger-Huet anomaly
C. pernicious anemia
B. autoimmune hemolytic anemia
D. sideroblastic anemia
Spherocytes are produced in autoimmune hemolytic anemia. Spherocytes may be produced by one of
three mechanisms. First, they are a natural morphological phase of normal red cell senescence.
Second, they are produced when the cell surface-to-volume ratio is decreased, as seen in hereditary
spherocytosis. And third, they may be produced as a result of antibody coating of the red cells. As the
antibody-coated red cells travel through the spleen, the antibodies and portions of the red cell
membrane are removed by macrophages. The membrane repairs itself; hence the red cells
morphology changes from a biconcave disk to spherocyte.
19. A patients peripheral smear reveals numerous NRBCs, marked variation of red cell morphology, and
pronounced polychromasia. In addition to a decreased hemoglobin and hematocrit values, what other
parameter would be consistent with these findings?
A. reduced platelets B. increased MCHC C. increased MCV
D. decreased RDW
The patients abnormal peripheral smear indicates marked red cell regeneration, causing many
reticulocytes to be released from the marrow. Since reticulocytes are larger than mature RBCs, the
MCV will be slightly elevated.

20. The classification of sickle cell trait versus sickle cell disease is based on the
A. severity of the clinical symptoms
C. level of compensatory hemoglobin F
B. number of irreversibly sickled cells
D. percentage of Hb S on electrophoresis
The number of irreversibly sickled cells (ISCs) and the proportion of S hemoglobin within the cells
contribute collectively to the severity of sickle cell disorders. The classification of trait versus disease
is not based on the severity of symptoms. The absence of Hb A and the presence of over 80% Hb S on
electrophoresis would be classified as homozygous sickle cell disease (SS), whereas the heterozygous
condition (AS) would show approximately 60% Hb A and 40% Hb S. Sickling is rare in the trait condition
because of the lower concentration of Hb S. Sickle cell disease typically shows increased levels of
compensatory Hb F, as does hemoglobin SC disease.
21. Which of the following statements about hereditary elliptocytosis (HE) is incorrect?
A. Characteristic oval shape occurs in mature erythrocytes.
B. Heterogenous group of disorders linked to Rh-null individuals.
C. Cellular defect involves the skeletal protein component of the membrane.
D. HE cells are abnormally permeable to sodium.
In hereditary elliptocytosis (HE), the red blood cells show increased permeability to sodium and may
have one of several membrane defects linked to this heterogenous disorder. These include deficiencies
in skeletal proteins such as protein 4.1 or spectrin. The characteristic oval or elliptical shape is seen
only in mature red blood cells, and it occurs in the circulation when HE red cells cannot return to a
normal biconcave shape.
22. Asynchronous nucleocytoplasmic maturation due to impaired DNA metabolism is a characteristic of
A. myelopthisic anemia
C. sideroblastic anemia
B. pure red cell aplasia
D. megaloblastic anemia
Deficiencies of folic acid (folate) and vit. B12 result in abnormal DNA synthesis and a resultant delay in
nuclear maturation in comparison to cytoplasmic development. These anemias are categorized as
megaloblastic because of the giant red cell precursors observed in the bone narrow.
23. Which of the following correctly describes punctuate basophilia?
A. lead deposits
B. DNA remnants
C. iron deposits
D. aggregates of residual RNA
Punctate basophilia or basophilic stippling is identified by the presence of RNA granules (coarse
stippling due to RNA instability in young RBCs, defective HbC or HbE synthesis, sideroblastic or
megaloblastic anaemia, thalassemia major and minor, preleukemic states and pyrimidine 5nucleotidase deficiency) and aggregates of precipitated ribosomes (fine stippling, resulting in diffuse
polychromasia secondary to increased RBC production in thalassemia, malabsorption and pernicious
anemia)
24. Which of the following erythrocyte inclusions represent remnants of mitotic spindles appearing as
thread-like strands in the shape of a loop usually seen in the blood of patients with myelodysplastic
syndrome?
A. Cabot rings
B. Heinz bodies
C. Dohle bodies
D. Howell-Jolly bodies
Cabot rings are thin, red-violet staining, thread-like strands in the shape of a loop or figure 8 that are
found on rare occasions in erythrocyte. They are believed to be microtubules that are remnants from a
mitotic spindle, and their presence indicates an abnormality in the production of red blood cells.
25. Which of the following RBC inclusions is associated with glucose-6-phosphate dehydrogenase
deficiency?
A. Howell-Jolly body B. Heinz body
C. Hb H body
D. Pappenheimer body
Heinz bodies are inclusions within red blood cells composed of denatured hemoglobin. G6PD (glucose6-phosphate dehydrogenase) deficiency exacerbated by administration of oxidant drugs (e.g.
primaquine, dapsone, quinidine) can also result in Heinz bodies. G6PD deficiency in red cells in
combination with high levels of oxidants causes a cross-linking of sulfhydryl groups on globin chains
which leads to denaturiation and formation of Heinz body precipitates.
26. The hematologic profile that is consistent with sickle cell disease includes all of the following except
A. neutrophilia and thrombocytosis
C. marked polychromasia on blood smear
B. increased osmotic fragility test
D. Howell-jolly bodies due to asplenia
Sickle cell disease is characterized by decreased osmotic fragility test.
27. A patient has a Hct of 0.30, a hemoglobin of 80 g/L, and an erythrocyte count of 4.0x10 12/L. What is the
morphological classification of this anemia?
A. normocytic, normochromic
C. macrocytic, hypochromic
B. microcytic, hypochromic
D. normocytic, hyperchromic
The indices will provide a morphological classification of this anemia. The MCV is 75 fL (reference
range 80-96 fL), the MCH is 20.0 pg (reference range 27-33), and the MCHC is 26.6% (reference range
33%-36%). Therefore, the anemia is microcytic hypochromic.
28. Which of the following hemoglobin electrophoresis results is most characteristic of -thalassemia trait?
A. Hb C is present
B. Hb A2 is elevated C. Hb S is elevated D. Hb H is present
The classical findings of a patient with -thalassemia trait are a microcytic hypochromic anemia with
mildly elevated RBC count and target cells on the peripheral blood smear. Basophilic stippling may also
be seen. Hemoglobin electrophoresis reveals increased HbA2 levels (up to around 9%). -thalassemia
is an anemia secondary to inadequate production of normal hemoglobin -chains. Hb H is an thalassemia in which three of the four -chain genes are either missing or non-functional. Hb S and Hb
C represent qualitative mutational changes in the -chain rather than quantitative changes.

29. Thalassemias are a group of congenital disorders characterized by


A. qualitative defects in globin structure
C. structural defects in the heme portion of hemoglobin
B. changes in hemoglobin solubility
D. quantitative defects in globin synthesis
Alpha-thalassemias result from gene deletions that cause a reduced rate of alpha-globin chain
production. Beta-thalassemias result from point mutations that cause a reduced rate of beta-globin
chain synthesis. Normally, equal amounts of alpha- and beta-globin chains are produced for Hb A
synthesis. In alpha- or beta- thalassemias, synthesis of globin chains is imbalanced, because a
decreased production rate of one type of globin chain causes an excess of the other.
30. Which of the following laboratory data are consistent with a diagnosis of pernicious anemia?
A. RBC = 2.5 x 1012/L; WBC = 12.5 x 109/L; PLT = 250 x 109/L
B. RBC = 4.5 x 1012/L ; WBC = 6.5 x 109/L; PLT = 150 x 109/L
C. RBC = 2.5 x 1012/L ; WBC = 2.5 x 109/L; PLT = 50 x 109/L
D. RBC = 3.0 x 1012/L ; WBC = 5.0 x 109/L; PLT = 750 x 109/L
Patients with pernicious anemia demonstrate a pancytopenia with low WBC, PLT, and RBC counts.
Because this is a megaloblastic process and a DNA maturation defect, all cell lines are affected. In the
bone marrow, this results in abnormally large precursor cells, maturation asynchrony, hyperplasia of all
cell lines, and a low M:E ratio.
31. A cellulose acetate electrophoresis revealed a large band of hemoglobin in the hemoglobin S position
quantified at 95%. The peripheral smear revealed 70% target cells, and the solubility test was negative.
Based on this information, what is the hemoglobin?
A. Hb S
B. Hb E
C. Hb C
D. Hb D
Hemoglobin D migrates in the same location as hemoglobin S on cellulose acetate at alkaline pH but
does not cause sickling. The negative solubility test rules out the presence of hemoglobin S. Target
cells are seen in large numbers in homozygous hemoglobin D disease. The quantification of 95%
differentiates homozygous from heterozygous states where less than 50% hemoglobin D would be
seen.
32. Which of the following statements about hemoglobin C disease is incorrect?
A. Lysine is substituted for glutamic acid at the 6th position of beta chain.
B. Target cells are frequently seen on peripheral smears.
C. Osmotic fragility of red cells is decreased.
D. Fast mobility of Hb C at pH 8.6.
Hemoglobin C disease results from a homozygous substitution of lysine for glutamic acid at the beta-6
position. Valine substitution results in sickle cell disease. Target cells and decreased osmotic fragility
are characteristic of hemoglobin C disease. Substitution of a positively charged amino acid for a
negatively charged amino acid in Hgb C disease results in a slow electrophoretic mobility at pH 8.6.
33. Autoimmune hemolytic anemia is best characterized by which of the following?
A. increased plasma C3
C. spherocytic red cells
B. decreased osmotic fragility
D. decreased unconjugated bilirubin
Spherocytes are characteristic of autoimmune hemolytic anemia and result in an increased osmotic
fragility. In autoimmune hemolytic anemias, production of autoantibodies against ones own red cells
causes hemolysis or phagocytic destruction of RBCs. A positive direct antiglobulin test identifies invivo
antibody-coated and complement-coated red cells. A positive DAT distinguishes AIHA from other types
of hemolytic anemia that produce spherocytes. Those AIHAs that produce complement binding
antibodies cause a depletion of C3. Those not associated with intravascular hemolysis cause an
increase in unconjugated bilirubin.
34. Which of the following is the primary Hgb in patients with thalassemia major?
A. Hb D
B. Hb F
C. Hb A
D. Hb C
Patients with thalassemia major are unable to synthesize the -chain; hence little or no Hb A is
produced. However, -chains continue to be synthesized and lead to variable elevations of Hb F in
these patients.
35. Iron deficiency anemia may be distinguished from anemia of chronic infection by
A. serum iron level
B. cell morphology
C. RBC indices
D. total iron-binding capacity
In iron deficiency anemia, the serum iron level is decreased and the total-iron binding capacity is
increased. In chronic disease the iron is trapped in reticuloendothelial cells and therefore is unavailable
to the red cells. Serum iron and TIBC are both decreased.
36. Select the amino acid substitution that is responsible for sickle cell anemia.
A. lysine for glutamic acid at the 6th position of the -chain
B. valine for glutamic acid at the 6th position of the -chain
C. valine for glutamic acid at the 6th position of the -chain
D. glutamine for glutamic acid at the 6th position of the -chain
The structural mutation for Hgb S is the substitution of valine for glutamic acid at the sixth position of
the -chain. Because glutamic acid is negatively charged, this decreases its rate of migration toward
the anode at pH 8.6.
37. What complication of sickle cell disease is more likely to occur in young adults with Hb SC than those
with Hb SS?
A. ischemic stroke
B. sickle retinopathy C. pulmonary hypertension D. acute coronary syndrome
All the complications listed except sickle retinopathy occur more frequently in Hgb SS patients.
Retinopathy occurs in both Hgb SS and Hgb SC patients, but it is more common in Hgb SC. The higher

total hemoglobin concentration, hence higher blood viscosity, is thought to contribute to this tendency.
38. All of the following are characteristics of megaloblastic anemia except
A. pancytopenia
C. elevated reticulocyte count
B. hypersegmented neutrophils
D. macrocytic erythrocyte indices
Megaloblastic anemias are associated with an ineffective erythropoiesis and therefore a decrease in
the reticulocyte count.
39. A screening test for hemoglobinopathies on a 4-month old baby showed a trace amount of Hb Barts in
addition to the normal Hb F and Hb A. This finding indicates that the baby has a form of
A. alpha thalassemia B. beta thalassemia C. gamma thalassemia
D. delta-beta thalassemia
Newborns make a predominance of fetal hemoglobin (Hb F) and a lesser amount of adult hemoglobin
(Hb A), giving the normal F, A pattern on newborn screening. Hb F is composed of 2 and 2 chains
(22). When there is a relative deficiency of alpha chains due to alpha thalassemia, then the relative
excess of unpaired gamma chains self-associate to form Hb Barts, a tetramer of gamma chains (4).
The presence of Hb Barts indicates the presence of alpha thalassemia.
40. A shift to the left, when used to describe a cell population, refers to
A. increased cells in the blood due to redistribution of blood pools
B. an increase in immature blood cells following release of bone marrow pools
C. a cell production hiatus or gap
D. a higher percentage of lymphocytes than neutrophils
A shift to the left means an increase in immature neutrophilic cells in the blood caused by bone marrow
release of cells in response to infection or tissue damage.
41. Qualitative and quantitative neutrophil changes in response to infection include the following except
A. neutrophilia
B. shift to the right
C. toxic granulation D. Dohle bodies
Neutrophil changes associated with infection may include neutrophilia, shift to the left characterized by
hyposegmentation, toxic granulation, Dohle bodies and vacuolization. The hyposegmentation in this
case must be differentiated from Pelgeroid hyposegmentation.
42. A patient with HIV is expected to have
A. shift to the left in WBCs B. target cells C. Pelgeroid Cells
D. reactive lymphocytes
HIV infections causes several hematological abnormalities which are usually seen on peripheral smear
examination and in most patients it demonstrate Reactive Lymphocytes and have granulocytopenia.
43. Which of the following is contained in the secondary granules of neutrophils?
A. lactoferrin
B. myeloperoxidase C. lysozyme
D. acid phophatase
Myeloperoxidase, lysozyme and acid phosphatase are enzymes that are contained in the primary
granules of neutrophil. The contents of secondary and tertiary granules include lactoferrin, collagenase,
NADPH oxidase, and alkaline phosphatase.
44. Chediak-Higashi syndrome is a disorder of neutrophil phagocytic function characterized by
A. pale blue cytoplasmic inclusions
C. nuclear hyposegmentation
B. giant lysosomal granules
D. small, dark-staining granules and condensed nuclei
Chediak-Higashi syndrome is caused by depressed chemotaxis and delayed degranulation. The
degranulation disturbance is attributed to interference from the giant lysosomal granules.
45. What percentage of neutrophils in the peripheral vasculature constitutes the marginal pool?
A. 100%
B. 80%
C. 50%
D. 30%
Approximately 50% of the neutrophils in the peripheral blood are found in the circulating pool. This is
the pool measured when a total WBC count is done. Another 50% are found adhering to vessel walls
(marginal pool). These pools are in constant exchange. Emotional or physical stimuli can cause a shift
of cells from the marginating pool to the circulating pool, causing a transient rise in the total WBC count.
The total WBC count can double but returns to normal within several hours.
46. Which of the following may be used to identify lymphocyte subtypes?
A. differences in nuclear shape
C. monoclonal antibody reactions to surface antigens
B. chromatin pattern in the nucleus
D. cytoplasmic granularity and overall cell size
Morphologic criteria such as cell size, nuclear shape and chromatin pattern or cytoplasmic granularity
cannot be used to identify lymphocyte subtypes. Monoclonal antibodies (CD surface markers) to
specific surface and cytoplasmic antigens can distinguish lymphocyte subpopulations and identify the
development stage. For example, blood lymphocytes that are B cells express CD19 and CD20
markers, T cells express CD2 and CD3 (and either CD4 and CD8 markers), and NK cells express
CD56. Natural killer cells often exhibit large granular lymphocyte morphology (LGLs).
47. What are the two most important products of the respiratory burst in neutrophils?
A. H2O2 and superoxide anion
C. lactoferrin and NADPH oxidase
B. cytochrome B and collagenase
D. alkaline phosphatase and ascorbic acid
The biochemical products of the respiratory burst that are involved with neutrophil particle ingestion
during phagocytosis are hydrogen peroxide and superoxide anion. The activated neutrophil discharges
the enzyme NADPH oxidase into the phagolysome, where it converts O2 to superoxide anion (O2-),
which is then reduced to hydrogen peroxide (H2O2).
48. What is the approximate amount of time a granulocyte spends in the circulation before migrating into
the tissues?
A. less than 1 day
B. about 3 days
C. up to 5 days
D. more than 10 days
After granulocytes are released from the bone marrow, they remain in the circulation for 1 day or less.

Their major function takes place in the tissues. They migrate through the vessel walls to reach areas of
inflammation very soon after release. The life span of the granulocyte is short; however, eosinophils
and basophils appear to survive longer in the tissues than the neutrophils.
49. Production of primary granules ceases and production of secondary granules commences with what
cell stage?
A. myeloblast
B. promyelocyte
C. myelocyte
D. metamyelocyte
The precursor cell that can first be recognized as granulocytic is the myeloblast and has no granules.
Primary or nonspecific granule production begins and ends during the promyelocyte stage. The
granules are distributed between daughter cells as mitotic divisions occur. Secondary or specific
granule production begins with the myelocyte stage and continues during succeeding cell stages with
the synthesis of products specific to the function of the particular granulocyteneutrophil, eosinophil, or
basophil.
50. A peripheral smear shows 75% blasts that stain positive for both Sudan black B and peroxidase. Which
of the following disorders is most likely?
A. AML
B. CML
C. AUL
D. ALL
AML blasts stain positive for Sudan Black B and peroxidase. Usually fewer than 10% blast are found in
the peripheral smear of patients with CML, unless there has been a transition to blast crisis. The
organelles in the cells of AUL are not mature enough to stain positive for SBB or Px. Blasts in ALL are
characteristically negative with these stains.
51. Which of the following statements about basophils is false?
A. membrane receptors bind IgE
C. morphologically resemble tissue mast cells
B. scanty water-soluble granules
D. granules contain heparin and anti-histamine
Basophils and tissue mast cells have receptors for IgE and complements, which trigger degranulation
when appropriate antigens are present and are responsible for severe hypersensitivity reactions
(anaphylaxis). Basophils and tissue mast cells have morphologic similarities but represent distinct cell
types. Basophils process water-soluble granules that contain, among other substances, heparin and
histamine (a vasodilator and smooth muscle contractor). Basophils have a segmented nucleus, and the
granules, although often scanty, overlie the nucleus. The mast cell has a single round nucleus, contains
many more granules than the basophil, and can be found in the bone marrow.
52. What does the granulocyte mitotic pool in the bone marrow contain?
A. myeloblasts and promyelocytes
C. band and segmented forms
B. the majority of marrow granulocytes
D. myelocytes and metamyelocytes
The granulocyte mitotic pool contains the cells capable of division, which are the myeloblasts,
promyelocytes, and myelocytes. The post-mitotic pool, or reserve, is the largest bone marrow pool and
contains metamyelocytes, band and segmented forms. This pool is available for prompt release into the
blood if needed (e.g., infection), and its early release is the cause of a left shift. If released, the bone
marrow mitotic pool can dramatically increase its activity to replenish this reserve (cytokine stimulation
increases).
53. Which of the following statements about leukocyte abnormalities is correct?
A. Hypersegmented neutrophils have four nuclear lobes
B. Auer rods are composed of fused primary granules.
C. Toxic granules are prominent secondary granules
D. Dhle bodies are agranular patches of DNA
Auer rods are seen in the cytoplasm of malignant cells, most often myeloblast, and are composed of
fused primary (nonseptic, azurophilic) granules. Hypersegmented neutrophils have five lobes or more
and are associated with vitamin B 12 or folate deficiency . Toxic granules are primary granules with
altered staining characteristics that stain in late -stage neutrophils due to toxicity . Dhle bodies are
agranular patches of RNA present in neutrophil cytoplasm and associated with toxic states.
54. Which of the following is a possible complication of infectious mononucleosis?
A. splenic infection B. hemolytic anemia C. giant platelets
D. leucopenia
Occasionally patients with infectious mononucleosis develop a potent cold agglutinin with anti-i
specificity. This cold autoantibody can cause strong hemolysis and a hemolytic anemia.
55. A female patient with massive splenomegaly was brought to the hospital. Her laboratory results showed
that she has mild anemia, a slightly elevated WBC count and a LAP score of 170. Her blood smear
showed tear drop erythrocytes and leukoerythroblastosis. These findings are more consistent with
A. primary polycythemia
C. chronic myelogenous leukemia
B. idiopathic myelofibrosis
D. primary thrombocythemia
The bone marrow is progressively replaced by fibrotic tissue in myelofibrosis, a chronic
myeloproliferative disorder. Attempts to aspirate bone marrow usually result in a dry tap. A biopsy
stain demonstrates increased fibrosis (fibroblasts are thought to be stimulated by megakaryocytes).
The presence of teardrop-shaped red blood cells is an important feature of myelofibrosis. In addition,
abnormal platelets, a leukoerythroblastic blood profile and myeloid metaplasia in the spleen and liver
are often associated with this disease. A high LAP score (Ref range 13-160) and increased RBC mass
are found in polycythemia vera, but the LAP score is low in chronic myelogenous leukemia.
56. Which of the following diseases is least likely to progress to acute leukemia?
A. refractory anemia with excess blasts
C. chronic lymphocytic leukemia
B. chronic myelogenous leukemia
D. refractory anemia with ringed sideroblasts
Progression to acute leukemia is a very unlikely event for patients with chronic lymphocytic leukemia,

even though there is no cure. Patients with chronic myelogenous leukemia typically progress to blast
crisis most often of myeloid type, unless treated with imatinibmesylate in the chronic phase. Refractory
anemia with excess blasts is the most likely type of myelodysplastic syndrome to develop acute
myelogenous leukemia. Refractory anemia with ringed sideroblasts is preleukemic but fairly stable.
57. Based on WHO classification system B cell ALL (FAB type L3) and _______ represent different clinical
presentations of the same disease entity.
A. Small lymphocytic lymphoma
C. Mycosis fungoides
B. Hodgkin lymphoma
D. Burkitt lymphoma
The lymphoid cells of B cell ALL FAB type L3 are morphologically identical to the malignant B cells of Burkitt
lymphoma (large cells with basophilic cytoplasm and cytoplasmic lipid vacuoles) although the site of origin is
the bone marrow in B cell ALL and the tissues in Burkitt lymphoma, the WHO classifies them as the same
disease entity with different clinical presentation. Both CLL and SLL are malignant proliferations of small,
mature lymphocytes, and diagnosis is based on the predominant site of involvement. Mycosis fungoides and
Sezary syndrome are different stages of a cutaneous T cell lymphoma in which the skin is the early sign of
involvement, with subsequent progression to the bone marrow and blood.
58. Which of the following is not among the diagnosic criteria used for classifying the myelodysplastic
syndromes?
A. hypocellular marrow with 25% blasts
C. hypogranular and hyposegmented neutrophils
B. presence of multinucleated red cells
D. unexplained and refractory anemia
Myelodysplastic syndromes are characterized by a hypercellular bone marrow and up to 20% marrow blasts
that distinguish MDS from acute leukemia. The blood and bone marrow blast percentages differ, and the risk
of transformation to acute leukemia varies with the types of MDSs. These disorders are characterized by
one or more peripheral blood cytopenias along with features of abnormal growth in the bone marrow. A
consistent feature in all types of myelodysplasia is unexplained and refractory anemia. Abnormalities may be
morphologic and/or functional. Criteria that help define the types of myelodysplastic syndromes include
megaloblastoid maturation of erythroid precursors, presence of multinucleated red cells, ringed sideroblasts,
hypogranular and/or hyposegmented neutrophils, monocytosis, abnormal platelet morphology, circulating
micromegakaryocytes, and degree of dyspoiesis.
59. Which of the following statements about non-Hodgkin types of lymphoma is true?
A. commonly associated with multiple bone lesions
B. initially present as a systemic disease rather than a localized tumor
C. lymphadenopathy is the most common presenting syndrome
D. characterized by proliferation of malignant cells primarily involving the bone marrow
Prominent lymphadenopathy is the most consistent finding in non-Hodgkin types of lymphoma at
presentation, but lymphoma may also arise in the spleen, liver or GI tract. Lymphomas begin as
localized tumors involving lymphoid tissue that spread to the bone marrow and blood. The malignant
lymphoid cells are immunologically classified as B cell or T N/K cell. Clonality can also be established
by demonstrating gene rearrangements via molecular analysis. Some common subtypes of nonHodgkin lymphoma are small lymphocytic, Burkitt, follicular, and mantle cell lymphomas. Leukemias are
initially systemic disorders primarily involving the bone marrow and blood at onset. Bone lesions are
associated with multiple myeloma.
60. What type of leukemia is characterized by the presence of numerous myeloblasts and an
abnormal proliferation of developing granulocytes and monocytes?
A. acute myeloblastic B. promyelocytic
C. myelomonocytic D.chronic myelogenous
Atypical promyelocytes predominate in promyelocytic leukemia. Myeloblasts predominate in AML. In
CML, there is predominance of not only myeloblasts and myelocytes but also metamyelocytes and
neutrophils.
61. A bone marrow with 90% cellularity and myeloid:erythroid (M:E) ratio of 10:1 is most
characteristic of
A. chronic myelogenous leukemia
C. polycythemia vera
B. beta thalassemia major
D. aplastic anemia
Hypercellular bone marrow and high M:E ratio are most characteristic of the excessive granulocyte
production that occurs in chronic myelogenous leukemia. Polycythemia vera typically has a
hypercellular marrow with panhyperplasia and a normal or low M:E ratio. Beta-thalassemia major is a
severe hemolytic anemia in which RBC hyperplasia of the marrow is pronounced and a low M:E ratio is
usual. Aplastic anemia is associated with a hypocellular marrow with reduction of all cell lines and
normal M:E ratio.
62. Which of the following findings rule out acute leukemia?
A. anemia
B. basophilia
C. immature cells
D. normal platelet count
Patients diagnosed with acute leukemia suffers from anemia, basophilia in the peripheral blood and
bone marrow, immature precursor or blast cells in the peripheral blood and marrow, NRBCs in the
peripheral blood and with thrombocytopenia.
63. What stain differentiates acute myelocytic leukemia from acute lymphocytic leukemia?
A. LAP
B. NASDA
C. peroxidase
D. methyl green pyronine
Acute myeloid leukemia gives a moderate to strongly positive reaction while Acute Lymphocytic
leukemia gives a negative reaction, this is due to the principle that in the presence of hydrogen

peroxide, myeloperoxidase in leukocyte granules oxidizes benzidine dihydrochloride from a colorless


form to a reddish brown derivative.
64. A peripheral blood smear demonstrating an absolute increase in small hypermature
lymphocytes and smudge cells would be suggestive of
A. CLL
B. ALL
C. IM
D. AML
CLL is twice as common in men compared with women, onset is slow and is commonly discovered in
the late stages of the disease. 80-90% of WBC are lymphocytes. Smudge cells are common and
hypogammaglobulinemia is present.
65. Which of the following may be used to confirm a diagnosis of hairy cell leukemia?
A. PAS
B. ACP
C. SBB
D. Unna pappenheim stain
One of the most important applications of acid phosphatase reaction is in classifying lymphoproliferative
disorders because T lymphocytes and monocytes stain positive. Tartrate-resistant acid phosphatase
(TRAP) is used to confirm a diagnosis of hairy cell leukemia.
66. What staining method is used most frequently to stain and count reticulocytes?
A. cytochemical
B. Romanowsky
C. supravital
D. immunofluorescence
The reticulum within thereticulocytes consists of ribonucleotides acid (RNA), which cannot be stained
with Wrights stain. Supravital staining with new methylene blue is used to identify the reticulocytes.
67. What is the normal WBC differential lymphocyte percentage (range) in the adult population?
A. 3-8%
B. 25-40%
C. 2-6%
D. 50-70%
The normal adult percentage of lymphocytes in a white cell differential is between 20% and 50%. The
range is higher in the pediatric population.
68. Autoagglutination of red cells at room temperature can result in which of the following?
A. low RBC count
B. high MCV
C. low Hct
D. all of these
Autoaggulitination at room temp causes a low RBC count and high MCV from an electronic counter.
The Hct will be low because it is calculated from the RBC count. Low RBC count and low Hct cause a
falsely high calvulations of MCH and MCHC.
69. What effect would using a buffer at pH 6.0 have on a Wrights stained smear?
A. red cells would be stained too blue
C. white cell cytoplasm would be stained too blue
B. red cells would lyse on the slide
D. red cells would be stained too pink
The pH of the buffer is critical in Romanowsky stains. When the pH is too low (<6.4), the red cells take
up more acid dye (eosin), becoming too pink. Leukocytes also show poor nuclear detail when the pH is
decreased.
70. What is the principle employed by automated impedance cell counters?
A. angle of laser beam scatter by cells
C. interruption of an electrical current by cells
B. amplification of electric current by cells D. hange in optical density of the solution containing cells
Blood cells are nonconductors of electrical current; they create a resistance/impedance of current in a
diluent solution that is conductive. When the suspension is forced through a small aperture, the current
flow is interrupted by the presence of the cells. A pulse is generated is proportional to the number of
pulses generated is proportional to the number of particles present; and the size and the size of the
pulse generated is proportional to the size of the cell.
71. What combination of reagents is used to measure hemoglobin?
A.sodium sulfate and sodium metabisulfate C. potassium ferricyanide and potassium cyanide
B.sodium citrate and hydrogen peroxide
D. hydrochloric acid and p-dimethyl aminobenzaldehyde
The standard assay for hemoglobin utilizes potassium ferricyanide and KCN. This solution, formerly
called Drabkins reagent, is now called cyanmethemoglobin (HiCN) reagent. The ferricyanide oxidizes
hemoglobin iron from ferrous (fe2+) to ferric (fe3+), and the potassium cyanide stabilizes the pigment as
cyanmethemoglobin for spectrophotometric measurement.
72. Which of the following is not source of error when measuring hemoglobin by the
cyanmethemoglobin method?
A. excessive antigoagulant
C. WBC count that exceeds linearity limits
B. lipemic plasma
D. scratched or dirty hemoglobin measuring cells
Anything that causes an increase in absorbance will cause the hemoglobin level to be falsely high. It is
necessary to correct for this type of error, such as making a plasma blank in the case of lipemia or
icterus. WBCs are present in the hemoglobin dilution and usually do not interfere. However, when the
WBC count is extremely high, their presence will cause cloudiness increasing the absorbance in the
hemoglobin concentration. Excessive anticoagulant does not affect hemoglobin readings.
73. Which of the following statements about reticulocyte counts is false?
A. The blood-stain mixture is incubated for 5-10 minutes.
B. New methylene blue, a supravital stain is used.
C. RBC inclusions can result in falsely elevated counts.
D. An erythrocyte must have at least four(4) blue particles to be counted as reticulocyte.
A living cell stain using new methylene blue is performed for reticulocyte counts. Reticulocytes should
not be stained for less than 5 minutes. Howell-Jolly bodies, Pappenheimer bodies, crenated cells and
refractile artifacts can be mistaken for reticulocyte inclusions. Two or more particles of reticulum
constitute a reticulocyte.
74. Which of the following tests could be performed on a hemolyzed blood sample?
A. hemoglobin only
C. hemoglobin and platelet count
B. RBC count and hematocrit
D. no results would be reportable

Hemoglobin is valid on a hemolyzed specimen, because RBC lysis is the first step in the
cyanmethemoglobin method. The red blood cell count depends on the presence of intact red blood
cells. Red blood cell fragments caused by hemolysis may be as small as platelets and affect
instruments that use sizing criteria to differentiate the two. Therefore, samples for these procedures
should be re-collected.
75. For which of the following procedures would heparin be recommended as anticoagulant?
A. platelet count
B. coagulation tests C. osmotic fragility
D. smear-based red cell morphology
Heparin is used for osmotic fragility and red cell enzyme studies, because it results in less lysis and
less membrane stress than other anticoagulants. Heparin induces platelet clumping and is
unacceptable for the platelet count. Heparin is unacceptable for coagulation test procedure because it
binds with antithrombin to neutralize many enzymes especially thrombin. This would cause very long
coagulation test results. EDTA is recommended for most routine hematology procedures, especially for
Wrights stained smears. Sodium citrate or EDTA can be used for sedimentation rates.
76. How would the following results on a 72 year old adult female be interpreted?
Hemoglobin - 6 g/dL
MCV - 114 fL
MCH - 39 pg
MCHC - 34 g/L
RDW - 15.0%
Reticulocyte count - 1.2%
Serum B12 - 55 pg/mL (N=200-1000)
Serum folate - 7 ng/ml (N=2-10)
Anti-IF antibodies - positive Schilling test: Part I - 5% excretion Part II (with IF) - 10% excretion
A. folate deficiency B. liver disease
C. pernicious anemia D. reticulocytosis
Oval macrocytes, increased B12, Positive IF antibodies and a Schilling test less than 7% on the first
day are all indicators of pernicious anemia.
77. Which of the following blood film findings indicate EDTA-induced pseudothrombocytopenia?
A. No platelets at all are seen on the film. C. The platelets are pushed to the feathery end.
B. The platelets are adhering to WBCs.
D. The slide has a bluish discoloration when examined.
Spuriously low platelet counts and falsely increased WBC counts (pseudoleukocytosis) can result from
EDTA-induced platelet clumping. Pseudoleukocytosis occurs when platelet agglutinates are similar to
WBCs and automated analyzers cannot distinguish the two. The platelet clumps are counted as WBCs
instead of platelets.
78. The production of wedge blood films that are too long and thin can be improved by
A. increasing the downward pressure on the spreader slide
B. decreasing the speed of spreading motion
C. placing the drop of blood closer to the center of the slide
D. increasing the angle between the spreader and stationary slides
The spreader slide held securely in the dominant hand at about 30-45 degree angle is drawn in back
into the drop of blood and the blood is allowed to spread across the width of the slide. The angle
determines the length of the smear. So does the speed of the spreader.
79. Which of the following erythrocyte inclusions can be visualized with supravital stain but cannot
be detected on a Wright-stained blood smear?
A. basophilic stippling B. Heinz Bodies
C. siderotic granules D. Howell-Jolly bodies
Heinz bodies are irregular, refractile, purple inclusions that are not visible with Wrights stain but show
up with supravital staining.
80. If patient has a reticulocyte count of 9% and an Hct of 29%, what is the corrected reticulocyte
count?
A. 5.6
B. 5.7
C. 5.8
D. 5.9
CRC= Reticulocyte (%) X [Hct (%) 45] , the average normal Hct. In this case 9 X (29 45) = 5.79996
81. 13 reticulocytes where counted in the large square of a calibrated Miller disc and 27 red blood
cells in the small square. The reticulocyte count is suggestive of
82. A. hemolytic anemia B. pernicious anemia C. aplastic anemia
D. megaloblastic anemia
83. Using a calibrated Miller disc the % Retics is computed by this formula: [# of reticulocytes
counted in large square (# of rbc counted in small square X 9)] X 100 . In this case the patient
has a reticulocyte count of [13 (27 X 9)] X 100 = 5.35 %, where in the normal value for adult is
0.5-1.0 %. Hemolytic anemia and blood loss anemia are some causes of high reticulocyte count
(%).
84. Using the Wongs method, a female patient had a hemoglobin level of 130 g/L. How much iron
does her hemoglobin contain?
A. 29.87 mg
B. 45.11 mg
C. 58.99 mg
D. 62.46 mg
Each gram per dL hemoglobin contains 3.47 mg of iron. Therefore, 13 X 3.47= 45.11 mg.
85. The white blood cell count, when the differential count has 4 nucleated red blood cells and the
automated analyzer white blood cell count is 9.7, is
A. 9.0
B. 9.1
C. 9.2
D. 9.3
Corrected WBC count = (Actual WBC count X 100) (# NRBC + 100). In this case, (9.7 X 100) (4 +
100) = 9.32 X 109 / uL .
86. In storage pool disease, platelets are primarily deficient in
A. ADP
B. platelet factor 3
C. thrombasthenin
D. thromboxane A2
Platelets in storage pool disease are deficient in dense granules. The platelets in this disorder lacks
nonmetabolic ADP found in dense granules and normally released when the platelets are stimulated.
This accounts for a poor response to aggregating agents.
87. Which of the following is associated with increased bleeding time and an abnormal platelet
aggregation test?

A. FVIII deficiency
B. FVIII inhibitor
C. Lupus anticoagulant
D. afibrinogenemia
Fibrinogen is a plasma protein that is essential for platelet aggregation and fibrin formation. In
afibrinogenmia both platelet function test (bleeding time and platelet aggregation) are abnormal.
88. A 25-year-old male presents to his physician complaining of leg pain. The physician diagnoses
a deep vein thrombosis and wants to determine the cause of the thrombotic episode. Which of
the following conditions should not be associated with such a thrombotic episode?
A. Factor V Leiden and Prothrombin 20210 mutations
B. Hypofibrinogenemia and hyperhomocysteinemia
C. Lupus anticoagulant and anticardiolipin antibodies
D. Antithrombin and protein C deficiencies
All of the conditions listed are associated with thrombosis except hypofibrinogenemia. Although
hereditary dysfibrinogenemia frequently causes thrombosis, hypofibrinogenemia cuases bleeding
tendencies. The most common hereditary thrombotic disorder factor V Leiden, is caused by synthesis
of an abnormal factor V molecule that is resistant to the inhibitory effects of protein C. the prothrombin
20210 mutation, in which an abnormal factor II molecule is synthesized, is the second most common
hereditary thrombotic disorder.
89. Which disorder maybe indicated given the following results?
PT= prolonged
APTT= prolonged
Platelet count= decreased Bleeding time= increased
A. FVIII deficiency
B. vWD
C. DIC
D. Factor IX deficiency
In DIC, this is a diffuse intravascular generation of thrombin and fibrin. As a result, coagulation factors
and platelets are consumed, resulting in decreased platelet count and decreased PT, APTT and
bleeding time.
90. Which set of platelet responses would be most likely associated with Glanzmanns
thrombasthenia?
A. Normal platelet aggregation to ADP and ristocetin; decreased aggregation to collagen
B. Normal platelet aggregation to collagen; decreased aggregation to ADP and ristocetin
C. Normal aggregation to ristocetin; decreased aggregation to collagen, ADP, and epinephrine
D. Normal platelet aggregation to ADP; decreased aggregation to collagen and ristocetin
Glanzmanns thrombasthenia is a disorder of platelet aggregation. Platelet aggregation is normal in
response to ristocetin but abnormal in response to collagen, ADP, and epinephrine.
91. Which of the following is a characteristic of acute idiopathic thrombocytopenic purpura?
A. predominantly seen in adults
C. insidious onset
B. nonimmune platelet destruction
D. spontaneous remission within a few weeks
Acute idiopathic thrombocytopenic purpura is an immune-mediated disorder found predominantly in
children. It is commonly associated with infection (primarily viral). It is characterized by abrupt onset,
and spontaneous remission usually occurs within several weeks.
92. TTP differs from DIC in that
A. schistocytes are not present in TTP but are present in DIC
B. APTT is normal in TTP but prolonged in DIC
C. platelet count is decreased in TTP but normal in DIC
D. PT is prolonged in TTP but decreased in DIC
In DIC, the PT and APTT are both prolonged, the platelet count is decreased, and schistocytes are
seen in the peripheral smear. Thrombotic thrombocytopenic purpura is a platelet disorder in which
platelet aggregation increases, resulting in thrombocytopenia. Schistocytes are present in TTP as a
result of microangiopathic haemolytic anemia; however, the PT and APTT are both normal.
93. Storage pool deficiencies are defects of
A. platelet granules B. platelet adhesion C. platelet aggregation
D. platelet production
Storage pool deficiencies are defects of platelet granules. Most commonly, a decrease in platelet dense
granules is present with decreased released ADP, ATP, calcium, and serotonin from platelet dense
granules.
94. Which of the following is correct regarding acquired thrombotic thrombocytopenic purpura?
A. decreased vWF
C. autoimmune disease
B. decreased platelet adhesion
D. decreased platelet aggregation
Acquired TTP is an autoimmune disease associated with autoantibodies produced against vWF
cleaving enzyme (ADAMTS-13). This deficiency results in an increase in plasma vWF and
consequently increased platelet aggregation and thrombocytopenia.
95. The extrinsic pathway of coagulation begins with the activation of _____ in the early stage.
A. phospholipoproteins
B. Factor VII C. Ca2+
D. tissue thromboplastin
The extrinsic pathway is initiated by the entry of Tissue Thromboplastin into the circulating blood.
Tissue thromboplastin is derived from phospholipoproteins and organelle membranes from disrupted
tissue cells.
96. The oral anticoagulant warfarin acts by
A. neutralizing the effects of thrombin
C. interfering with fibrin monomer formation
B. acting as a vitamin K antagonist
D. inducing hypercoagulation
It is a traditional oral anticoagulant that interferes with the normal synthesis of FactorsII, VII, IX and X
as well as protein C and S.
97. Which of the following is characteristi of protein C?
98. A. not Vitamin K dependent
C. formed in response to thrombin generation

B. inactivates factors Va and VIIIa


D. none of these
Protein C, a vitamin Kdependent plasma protein synthesized in the liver, it represents anatural
anticoagulant formed in response to thrombin generation.
99. If a child ingested rat poison, which of the following tests should be performed to test the effect of the
poison on the childs coagulation mechanism?
A. APTT
B. PT
C. fibrinogen assay D. thrombin time
Prolonged values of PT will be seen if an oral anticoagulant such as coumarin or a coumarincontaining substance, such as rat poison, is ingested.
100. Which of the following is not synthesized in the liver?
A. antihemophilic factor
B. protein C C. plasminogen
D. von Willebrand factor
The liver produces most of the clotting factors as well as inhibitors to clotting. A patient with liver
disease has impaired synthesis of these clotting factors and inhibitors. One of the few hemostatic
proteins not produced by the liver is von Willebrand factor, which is produced by endothelial cells and
megakaryocytes.
101. When thrombin binds to thrombomodulin on the endothelial cell surface, thrombin can
A. activate the protein C pathway
C. convert fibrinogen to fibrin
B. stimulate platelet aggregation
D. activate factor V and factor VIII
Thrombomodulin, an endothelial cell receptor, has the ability to change the specificity of thrombin from
a procoagulant to an anticoagulant. Once bound to thrombomodulin, thrombin has anticoagulant
properties because of its activation of protein C. Protein C, along with its cofactor protein S, then
exerts negative feedback on the clotting system by inactivating factor V and factor VIII.
102. Which of the following complexes is not needed for blood coagulation to occur?
A. Xa, V, Ca2+, PF3
C. VIIa, tissue factor, Ca2+
B. XIIa, kallikrein, HMWK
D. Xa, VIII, Ca2+, PF3
The intrinsic, extrinsic and common pathway each have a complex that must form for blood
coagulation to occur. The intrinsic complex of IXa, VIII, and Ca2+ forms on the platelet surface (PF3)
and activates factor X. The extrinsic complex of VIIa, tissue factor, and Ca2+ activates factor X as well
as factor IX and is the dominant pathway in vivo. Factor IX activation by the extrinsic complex provides
a link between the extrinsic and intrinsic systems and minimizes the importance of the contact factors
in vivo. The prothrombin-converting complex of Xa, V, Ca2+, and PF3 is responsible for converting
prothrombin to thrombin.
103. Von Willebrand factor is a
A. plasma protein that binds platelets to exposed subendothelial collagen
B. plasma protein with procoagulant activity in the intrinsic coagulation system
C. phospholipid required for multiple reactions in the coagulation sequence
D. platelet membrane glycoprotein that attaches the platelet to the injured vessel wall
vWF is a portion of the plasma protein known as the factor VIII/von willebrand factor complex. Its
function is to bind to platelet membrane glycoprotein Ib and form a bridge between the platelet and
exposed subendothelial collagen. vWF is a carrier protein for factor VIII:C, but vWF does not have
coagulant activity in secondary hemostasis as factor VIII:C does.
104. The coagulation factors referred to as vitamin K-dependent include
A. I, V, VIII, XIII
B. II, V, IX, XII
C. II, VII, IX, X
D. XI, XII, Fletcher, Fitzgerald
The prothrombin group factors II, VII, IX, and X are called vitamin K-dependent factor. Vitamin K is
needed by the liver to synthesize functional circulating forms of these factors. In the absence of
vitamin K, the liver synthesizes the prothrombin group factors, but they are nonfunctional because they
lack the carboxyl (COOH) groups needed for binding to Ca2+ on phospholipid membranes. The oral
anticoagulant warfarin is a vitamin K antagonist and causes liver synthesis of these nonfunctional
factors.
105. The main regulatory protein of secondary homeostasis is
A. protein C
B. antithrombin
C. 2-Antiplasmin
D. tissue plasminogen activator
Antithrombin is the most important naturally occurring inhibitor toclotting and accounts for 80% of
negative feedback in the coagulation cascade by inhibiting serine proteases. Protein C and its
cofactor, protein S, inhibit cofactor V and VIII. A2-antiplasmin is responsible for neutralizing plasmin
once the clot has been lysed. Tissue plasminogen activator activates the fibrinolytic system in
response to clot formation.
106. Factor VIII has the highest concentration in?
A. FFP
B. whole blood
C. cryoprecipitate
D. platelet concentrate
Cryoprecipitate is a source of fibrinogen. Fibrinogen is vital to blood clotting. It is usually used in the
treatment of patients with reduced levels of, or poorly functioning, fibrinogen with clinical bleeding, an
invasive procedure or trauma.
107. Which of the following is (are) true of protein S?
A. It is a cofactor of protein C
B. It increases the rate of inactivation of factor Va
C. It enhances the binding of APC to phospholipids
D. Both A and B
Protein S is a Vitamin-K dependent plasma protein that is an essential cofactor for APC to express
anticoagulant effect. It increases the rate of inactivation of factor Va by APC by enhancing the binding
of APC to phospholipids, thereby stimulating the inactivation of factor Va.

108. The following are rendered inactive by coumarin anticoagulants except


1. Factor XI
2. Factor VII
3. FactorVIII
4. Factor IX
A. 1 and 3
B. 2 and 4
C. 1,2,and 3
D. 1, 2, 3, 4
Coumarin anticoagulants have antagonistic effect on Vitamin K dependent coagulation factors since it
inhibits Vitamin K reduction from the epoxide form. These drugs cause incomplete
coagulationbecause they lack calcium-binding sites and cannot form enzyme substrate complexes.
The end result is that the Factors II, VII, IX and X are rendered inactive.
109. Which of the following laboratory results are not consistent with Factor VII deficiency?
1. Abnormal Stypven time 2. Normal bleeding time
3.Normal PT
4. Normal TT
A. 1 and 3
B. 2 and 4
C. 1, 2, and 3
D. 1,2,3,4
PT tests are prolonged in Factor VII deficiency since it measures the production of fibrin via the
extrinsic and common pathways in which factor VII is necessary. However, the addition of Russels
viper venom (Stypven time) allows activation of Factor X directly, bypassing Factor VII as a necessary
component and therefore renders the Stypven time to be unaffected by Factor VII deficiency.
110. Factor IX deficiency can be treated using
A. Factor VIII concentrate
C. cryoprecipitate
B. FFP replacement therapy
D. prothrombin complex concentrate
FFP replacement therapy is the treatment of choice for Factor IX deficiency since FFP contains active
factor IX.
111. A prolonged APTT and PT are corrected when mixed with normal plasma. Which factor is most likely
deficient?
A. VIII
B. IX
C. XI
D. V
Factor V, a common pathway factor deficiency, is most likely suspected, since both PT and APTT are
prolonged and both are corrected when mixed with normal plasma.
112. A prolonged APTT is corrected with factor VIII-deficient plasma but not with factor XI-deficient plasma.
Which factor is deficient?
A. V
B. IX
C. VIII
D. X
Since the prolonged APTT is not corrected with a factor IX-deficient plasma, factor IX is suspected to
be deficient in the test plasma.
113. Which of the following is a characteristic of classic Hemophilia A?
A. prolonged PT
C. prolonged bleeding time
B. mild to severe bleeding episodes
D. autosomal recessive inheritance
Hemophilia A is characterized by mild to severe bleeding episodes, depending upon the concentration
of factor VIII:C . Hemophilia A is inherited as a sex-linked disease. Bleeding time and prothrombin time
are both normal in Hemophilia A.
114. The following laboratory results have been obtained from a 40-year-old woman: PT= 20 sec; APTT=
50 sec; thrombin time= 18 sec. What is the most probable diagnosis?
A. hypofibrinogenemia
C. Factor VIII deficiency
B. Factor VII deficiency
D. Factor X deficiency
Fibrinogen is a clotting protein of the common pathway and is evaluated by the thrombin time. In
hypofibrinogenemia (fibrinogen concentration <100 mg/dL), the PT, APTT, and TT are prolonged. In
factor VII deficiency, APTT is normal; in factor VIII deficiency the PT is normal; and in factor X
deficiency the TT is normal.
115. A protein that plays a role in both coagulation and platelet aggregation is
A. Factor I
B. Factor V
C. Factor X
D. Factor XII
Factor I is necessary for platelet aggregation along with the glycoprotein IIb/IIIa complex. Factor I is
also a substrate in the common pathway of coagulation. Thrombin acts on fibrinogen to form fibrin
clots.
116. Normal PT and APTT results in a patient with a poor wound healing may be associated with
A. Factor VII deficiency
C. Factor VIII deficiency
B. Factor XIII deficiency
D. Factor XII deficiency
Factor XIII deficiency can lead to impaired wound healing and may cause severe bleeding problems.
Factor XIII is a fibrin stabilizing factor that changes the fibrinogen bonds in fibrin polymers to stable
covalent bonds. Factor XIII is not involved in the process of fibrin formation and, therefore, the PT and
APTT are both normal.
117. Prolonged bleeding time and giant platelets best describe
A. Glanzmann thrombasthemia
C. Bernard-Soulier Syndrome
B. Von Willebrand disease
D. Wiskott-Aldrich syndrome
Options A and C are incorrect because platelet morphology is normal even though the bleeding time is
prolonged. Option D is incorrect because this syndrome is characterized by tiny platelets and a
prolonged bleeding time.
118. Which of the following would one suspect in afibrinogenemia?
A. Bleeding time normal, reptilase time abnormal, thrombin time abnormal
B. Bleeding time abnormal, reptilase time normal, thrombin time abnormal
C. Bleeding time normal, reptilase time normal, thrombin time normal
D. Bleeding time abnormal, reptilase time abnormal, thrombin time abnormal
Bleeding time, reptilase time and thrombin time are all sensitive to deficiencies in fibrinogen.
119. A 22-year old female was seen in the emergency room with evidence of bleeding following a spider

bite. Laboratory results show:


Blood smear: schistocytes Plt ct: 50,000/mm3 PT: 20 sec
aPTT: 60 sec D-dimer: positive
The most likely diagnosis is
A. allergic reaction
C. vitamin K deficiency
B. primary fibrinolysis
D. secondary fibrinolysis
Option A is incorrect as allergies do not result in coagulation abnormalities. Option C is incorrect
because schistocytes, decreased platelets and a positive D-dimer test are not seen in primary
fibrinolysis. Option B is not characterized by schistocytes and a positive D-dimer test.
120. Which of the following is an immune-mediated condition characterized by a low platelet count and is
found primarily in children?
A. May-Hegglin
C. idiopathic thrombocytopenia purpura
B. Wiscott-Aldrich
D. thrombotic thrombocytopenia purpura
ITP is an immune-mediated disorder characterized by an abrupt onset and spontaneous remission in
several weeks. It is found predominantly in children. Option D is a non-immune-mediated condition
usually characterized by microangiopathic anemia, thrombocytopenia, fever, renal disease and
neurologic abnormalities. Option A is a non-immune-mediated condition characterized by
thrombocytopenia (decreased platelet production) with small hypogranular platelets. Option B is an
immunologic condition characterized by immune deficiency (T and B cells). Thrombocytopenia with
severly shortened platelet survival and platelets with absent dense bodies are also associated with this
disease.
121. Thrombotic thrombocytopenic purpura is characterized by
A. prolonged PT
C. increased platelet aggregation
B. thrombocytosis
D. prolonged APTT
TTP is a quantitative platelet disorder associated with increased intravascular platelet activation and
aggreagation resulting in thrombocytopenia. The PT and APTT results are normal in TTP.
122. Which of the following prevents platelet aggregation?
A. thromboxane A2 B. thromboxane B2 C. prostacyclin
D. antithromin
Prostacyclin is released from the endothelium and is an inhibitor of platelet aggregation.
123. Thrombocytopenia may be associated with
A. postsplenectomy B. hypersplenism
C. acute blood loss D. proliferation of pluripotent cells
Hypersplenism is associated with thrombocytopenia. In this condition, up to 90% of platelets can be
sequestered in the spleen, causing decreases in circulatory platelets.
124. Which of the following test results is not characteristic of DIC?
A. decreased fibrinogen concentration
C. positive test for degradation products
B. decreased platelet count
D. increased anti-thrombin
The PT and APTT are prolonged in DIC because of consumption of factors I, II, V, VIII. Platelets are
trapped in forming clots and are removed from the circulation. The fibrinolytic system is activated by
systemic intravascular coagulation; fibrin and fibrinogen degradation products are elevated.
125. Aspirin prevents platelet aggregation by inhibiting the action of which enzyme?
A. phospholipase
B. cyclooxygenase C. thromboxane A2 D. prostacylin synthetase
Aspirin prevents platelet aggregation by inhibiting the activity of the enzyme cyclo-oxygenase. This
inhibition prevents the formation of thromboxane A2 (TXA2), which serves as a potent platelet
aggregator.
126. Which defect characterizes Gray syndrome?
A. platelet adhesion defect
C. dense granule defect
B. alpha granule defect
D. coagulation defect
Grays syndrome is a platelet granule defect associated with a decrease in alpha granules resulting in
decreased production of alpha granule proteins such as platelet factor 4 and beta thromboglobulin.
Alpha granule deficiency results in the appearance of agranular platelets when viewed on a wrights
stained blood smear.
127. Prekallikrein deficiency is associated with
A. prolonged aPTT that does not correct with a mixing study
B. increased risk of thrombosis
C. delayed bleeding at the incision site following surgery
D. autosomal dominant inheritance
Prekallikrein (Fletcher factor) deficiency is one of the many autosomal recessive disorders. The aPTT
will be prolonged and will correct with a mixing study because it is a factor deficiency. Because
prekallikrein is an activator of the fibrinolytic system, prekallikrein-deficient patients cannot lyse clots
efficiently and are prone to thrombosis. Fibrinolytic and anticoagulant therapies are indicated in
patients who develop thrombosis. Delayed post-operative bleeding at the incision site is characteristic
of a factor XIII deficiency.

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