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HEMATOLOGY-MTAP > CHAPTER 19 > Flashcards

CHAPTER 19 Flashcards

1
Q
  1. 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

A

c. Fatigue, infection, and bleeding

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2
Q
  1. Idiopathic acquired aplastic anemia is due to a(n):

a. Drug reaction
b. Benzene exposure
c. Inherited mutation in stem cells
d. Unknown cause

A

d. Unknown cause

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3
Q
  1. The pathophysiologic mechanism in acquired idiosyncratic aplastic anemia is:

a. Replacement of bone marrow cells by
abnormal cells
b. Destruction of stem cells by autoimmune T cells
c. Defective production of hematopoietic
growth factors
d. Inability of bone marrow stroma to support stem cells

A

b. Destruction of stem cells by
autoimmune T cells

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4
Q
  1. Based on the criteria in Table 19.1, what is the aplastic anemia classification of a 15-year-old girl with a bone marrow cellularity of 10%, hemoglobin of 7 g/dL, absolute neutrophil count of 0.1 3 109 /L, and
    platelet count of 10 3 109 /L?

a. Nonsevere
b. Moderate
c. Severe
d. Very severe

A

d. Very severe

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5
Q
  1. The most consistent peripheral blood findings in severe aplastic anemia are:

a. Hairy cells, monocytopenia, and
neutropenia
b. Macrocytosis, thrombocytopenia, and
neutropenia
c. Blasts, immature granulocytes, and
thrombocytopenia
d. Polychromasia, nucleated RBCs, and
hypersegmented neutrophils

A

b. Macrocytosis, thrombocytopenia, and
neutropenia

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6
Q
  1. 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. Bone marrow transplant with an
HLA-identical sibling

A

d. Bone marrow transplant with an
HLA-identical sibling

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7
Q
  1. The test that is most useful in differentiating Fanconi anemia 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

c. Diepoxybutane-induced chromosome
breakage

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8
Q
  1. 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

A

d. Premature death of hematopoietic
stem cells

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9
Q
  1. Diamond-Blackfan anemia differs from Fanconi 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

A

c. Only erythropoiesis is affected

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10
Q
  1. 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

A

d. Congenital dyserythropoietic anemia

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11
Q
  1. RBC destruction that occurs when macrophages ingest and destroy RBCs is termed:

a. Extracellular
b. Macrophage mediated
c. Intra-organ
d. Extrahematopoietic

A

b. Macrophage mediated

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12
Q
  1. 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

A

d. Elevated urinary urobilinogen level

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12
Q
  1. The primary pathophysiologic mechanism of anemia associated with chronic kidney disease is:

a. Inadequate production of
erythropoietin
b. Excessive hemolysis
c. Hematopoietic stem cell mutation
d. Toxic destruction of stem cells

A

a. Inadequate production of
erythropoietin

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12
Q
  1. Which one of the following findings is not consistent with myelophthisic anemia?

a. Reticulocytosis
b. Teardrop RBCs
c. Extramedullary hematopoiesis
d. Leukoerythroblastic blood picture

A

a. Reticulocytosis

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13
Q
  1. The term hemolytic disorder in general refers to a disorder in which there is:

a. Increased destruction of RBCs in the
blood, bone marrow, or spleen
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

A

a. Increased destruction of RBCs in the
blood, bone marrow, or spleen

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14
Q
  1. 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

A

b. Yellow skin and eyes

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15
Q
  1. A patient has anemia that has been worsening over the last several months. The hemoglobin level has been declining slowly, with a drop of 1.5 g/dL of hemoglobin over about 6 weeks. Polychromasia and anisocytosis are seen on the peripheral blood film, consistent with the elevated reticulocyte count and
    red cell distribution width (RDW). Serum levels of total bilirubin and indirect fractions are normal. The urinary urobilinogen level also is normal. When
    these findings are evaluated, the conclusion is drawn that the anemia does not have a hemolytic component. Based on the data given here, why was hemolysis ruled out as the cause of the anemia?

a. The decline in hemoglobin is too gradual
to be associated with hemolysis.
b. The elevation of the reticulocyte count
suggests a malignant cause.
c. Evidence of increased protoporphyrin
catabolism is lacking.
d. Elevated RDW points to an anemia of
decreased production.

A

c. Evidence of increased protoporphyrin
catabolism is lacking.

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15
Q
  1. Which of the following tests provides a good indication of accelerated erythropoiesis?

a. Urine urobilinogen level
b. Hemosiderin level
c. Reticulocyte count
d. Glycated hemoglobin level

A

c. Reticulocyte count

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16
Q
  1. A 5-year-old girl was seen by her physician several days before the current visit and was diagnosed with pneumonia. She was prescribed a standard course of antibiotics. Her mother has brought her to the physician again because the girl’s urine began to darken after the first visit and now is alarmingly dark. The girl has no history of anemia, and there is no family history of any hematologic disorder. The CBC shows a mild anemia, polychromasia, and a few schistocytes. This anemia could be categorized as:

a. Acquired, fragmentation
b. Acquired, macrophage mediated
c. Hereditary, fragmentation
d. Hereditary, macrophage mediated

A

a. Acquired, fragmentation

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17
Q
  1. 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 peripheral blood film. Spherocytes are also noted. Which one 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 blood
film

A

d. Spherocytes on the peripheral blood
film

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18
Q
  1. Under normal circumstances, the major fraction of bilirubin in the plasma is:

a. Unconjugated bilirubin secreted by in the
liver
b. Urobilinogen reabsorbed from the
intestines
c. Macrophage-secreted indirect bilirubin
d. Direct bilirubin conjugated by hepatocytes

A

c. Macrophage-secreted indirect bilirubin

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19
Q
  1. Which of the following sets of test results is typically expected with chronic fragmentation hemolysis? Serum Haptoglobin, Urine Hemoglobin,
    Urine Sediment, Prussian Blue Stain:

a. Increased Positive Positive
b. Decreased Negative Negative
c. Decreased Positive Positive
d. Increased Positive Negative

A

c. Decreased Positive Positive

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20
Q
  1. Which one of the following sets of results is consistent with HS?

a. Decreased osmotic fragility, negative DAT
result
b. Decreased osmotic fragility, positive DAT
result
c. Increased osmotic fragility, negative
DAT result
d. Increased osmotic fragility, positive DAT
result

A

c. Increased osmotic fragility, negative
DAT result

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20
Q
  1. In HS, a characteristic abnormality in the CBC results is:

a. Decreased MCH
b. Decreased platelet and WBC counts
c. Increased MCHC
d. Increased MCV

A

c. Increased MCHC

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21
Q
  1. The altered shape of the spherocyte in HS is due to:

a. Abnormal precipitation of the hemoglobin molecule
b. A mutated RBC membrane protein
affecting vertical protein interactions
c. A mutated RBC membrane protein
affecting horizontal protein interactions
d. Defective RNA catabolism and clearance

A

b. A mutated RBC membrane protein
affecting vertical protein interactions

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22
Q
  1. The RBCs in HE are abnormally shaped and have unstable cell membranes as a result of:

a. Defects in horizontal membrane
protein interactions
b. Deficiency in cation pumps in the RBC
membrane
c. Lack of Rh antigens in the RBC
membrane
d. Mutations in the ankyrin complex

A

a. Defects in horizontal membrane
protein interactions

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23
Q
  1. The peripheral blood film for patients with mild HE is characterized by:

a. Densely stained RBCs with a few irregular projections
b. Elliptical RBCs
c. Oval RBCs with one or two transverse
ridges
d. Overhydrated RBCs with oval central
pallor

A

b. Elliptical RBCs

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24
Q
  1. Laboratory test results for patients with HPP include all of the following except:

a. Increased MCV and normal RDW
b. Low fluorescence when incubated with
eosin-59-maleimide
c. Marked poikilocytosis with elliptocytes,
RBC fragments, and microspherocytes
d. RBCs that show marked thermal
sensitivity at 41° C to 45° C

A

a. Increased MCV and normal RDW

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25
Q
  1. Acanthocytes are found in association with:

a. Abetalipoproteinemia
b. G6PD deficiency
c. Rh deficiency syndrome
d. Vitamin B12 deficiency

A

a. Abetalipoproteinemia

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26
Q
  1. The most common manifestation of G6PD deficiency is:

a. Acute hemolytic anemia caused by
drug exposure or infections
b. Chronic hemolytic anemia caused by cell
shape change
c. Chronic hemolytic anemia caused by
intravascular RBC lysis
d. Mild compensated hemolysis caused by
ATP deficiency

A

a. Acute hemolytic anemia caused by
drug exposure or infections

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27
Q
  1. 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 3 days after the hemolytic episode began. How will this affect the test result?

a. Absence of enzyme activity
b. False decrease in enzyme activity due to
hemoglobinemia
c. False increase in enzyme activity due
to reticulocytosis
d. No effect on enzyme activity

A

c. False increase in enzyme activity due
to reticulocytosis

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28
Q
  1. The most common defect or deficiency in the anaerobic glycolytic pathway that causes chronic HNSHA is:

a. Glucose-6-phosphate dehydrogenase
deficiency
b. Lactate dehydrogenase deficiency
c. Methemoglobin reductase deficiency
d. Pyruvate kinase deficiency

A

d. Pyruvate kinase deficiency

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29
Q
  1. Which of the following laboratory tests would be best to confirm PNH?

a. Acidified serum test (Ham test)
b. Flow cytometry for detection of
eosin-59-maleimide binding on erythrocytes
c. Flow cytometry for FLAER binding,
CD24 on granulocytes, and CD14 on
monocytes
d. Osmotic fragility test

A

c. Flow cytometry for FLAER binding,
CD24 on granulocytes, and CD14 on
monocytes

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30
Q
  1. A 22-year-old man with a moderate decrease in hemoglobin level and a decrease in RBC, WBC, platelet, and reticulocyte counts has a history of
    infrequent and mild episodes of hemolysis with hemoglobinuria. His bone marrow showed 15% cellularity with no abnormal cells, and flow cytometry revealed that 15% of his circulating granulocytes were GPI deficient. He most likely has:

a. A hereditary RBC membrane defect
b. Classic PNH
c. Hypoplastic PNH
d. Subclinical PNH

A

c. Hypoplastic PNH

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31
Q
  1. 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

A

c. Intravascular RBC fragmentation

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32
Q
  1. 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

A

a. Schistocytosis and thrombocytopenia

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33
Q
  1. 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 ADAMTS13
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

A

b. Formation of platelet-VWF thrombi due
to autoantibody inhibition of ADAMTS13

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34
Q
  1. 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

A

b. Prothrombin time and partial
thromboplastin time

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35
Q
  1. Which of the following laboratory results may be seen in both traumatic cardiac hemolytic anemia and exerciseinduced hemoglobinuria?

a. Schistocytes on the peripheral blood film
b. Thrombocytopenia
c. Decreased serum haptoglobin
d. Hemosiderinuria

A

c. Decreased serum haptoglobin

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36
Q
  1. 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

A

c. P. knowlesi

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37
Q
  1. 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

A

d. Schistocytes and microspherocytes

38
Q
  1. 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 3 109 /L, a hemoglobin level of 10.5 g/dL, a platelet count of 134 3 109 /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

A

c. Babesiosis

38
Q
  1. 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

A

b. Allo- or autoantibody against an
RBC antigen

39
Q
  1. A 36-year-old woman was brought to the
    emergency department by her husband because she had experienced a seizure. He reported that she had been well until that morning, when she complained of a sudden headache and malaise. She was not
    taking any medications and had no history of previous surgery or pregnancy. Laboratory studies showed a WBC count of 15 3 109 /L, a hemoglobin level of 7.8 g/dL, a platelet count of 18 3 109 /L, and
    schistocytes and helmet cells on the peripheral blood film. Chemistry test results included markedly elevated serum lactate dehydrogenase activity and a
    slight increase in the level of total and indirect serum bilirubin. The urinalysis results were positive for protein and blood, but there were no RBCs in the urine sediment. Prothrombin time and partial
    thromboplastin time were within the reference interval. When the entire clinical and laboratory picture is considered, which of the following is the most likely diagnosis?

a. HUS
b. HELLP syndrome
c. TTP
d. Exercise-induced hemoglobinuria

A

c. TTP

39
Q
  1. Which of the following laboratory test results are abnormal in HELLP syndrome but not in DIC?

a. Aspartate aminotransferase
b. Prothrombin time
c. Platelet count
d. Hemoglobin

A

a. Aspartate aminotransferase

40
Q
  1. 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 a

A

d. Demonstration of IgG a

40
Q
  1. 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

A

a. Spherocytes

41
Q
  1. The pathophysiology of immune hemolysis with IgM antibodies always involves:

a. Complement
b. Autoantibodies
c. Abnormal hemoglobin molecules
d. Alloantibodies

A

a. Complement

42
Q
  1. In autoimmune hemolytic anemia, a
    positive DAT is evidence that an:

a. IgM antibody is in the patient’s
serum
b. IgG antibody is in the patient’s
serum
c. IgM antibody is sensitizing the
patient’s red blood cells
d. IgG antibody is sensitizing the
patient’s red blood cells

A

d. IgG antibody is sensitizing the
patient’s red blood cells

43
Q
  1. Which of the following is not a
    mechanism of drug-induced hemolytic
    anemia?

a. Drug adsorption on red blood cell
membrane
b. Drug-RBC membrane protein
immunogenic complex
c. RBC autoantibody induction
d. IgM autoantibody sensitization
of RBCs after exposure to cold
temperatures

A

d. IgM autoantibody sensitization
of RBCs after exposure to cold
temperatures

44
Q
  1. Which of the following describes a
    penicillin-induced AIHA?

a. Extravascular hemolysis,
positive DAT with IgG, gradual
anemia
b. Intravascular, possible renal failure,
positive DAT with C3d
c. Extravascular hemolysis, positive
DAT with C3d, acute onset d.
Intravascular hemolysis, positive DAT
with IgG
d. Demonstration of IgG and/or C3d
on the RBC surface

A

a. Extravascular hemolysis,
positive DAT with IgG, gradual
anemia

45
Q
  1. Which one of the following statements is
    true about DHTR?

a. It usually is due to an ABO
incompatibility
b. Hemoglobinemia and
hemoglobinuria often occur
c. It is due to an anamnestic
response after repeat exposure to
a blood group antigen
d. The DAT yields a positive result
for C3d only

A

c. It is due to an anamnestic
response after repeat exposure to
a blood group antigen

45
Q
  1. A 63-year-old man is being evaluated
    because of a decrease in hemoglobin of 5
    g/dL after a second cycle of fludarabine
    for treatment of chronic lymphocytic
    leukemia. The patient’s DAT result is
    strongly positive for IgG only, and antibody
    testing on his serum and an eluate of his
    RBCs yield positive results with all panel
    cells and the patient’s own cells. This
    suggests which mechanism of immune
    hemolysis for this patient?

a. Drug-RBC membrane protein
complex
b. Drug adsorption
c. RBC autoantibody induction
d. Drug-induced nonimmunologic
protein adsorption

A

c. RBC autoantibody induction

46
Q
  1. Chronic secondary CAD is most often
    associated with:

a. Antibiotic therapy
b. M. pneumoniae infection
c. B cell malignancies
d. Infectious mononucleosis

A

c. B cell malignancies

46
Q
  1. A group A Rh-negative mother gave birth to a group O Rh-positive baby. The baby is at risk for HDFN if:

a. This was the mother’s first
pregnancy
b. The mother has IgG ABO
antibodies
c. The mother was previously
immunized to the D antigen
d. The mother received Rh immune
globulin before delivery

A

c. The mother was previously
immunized to the D antigen

47
Q
  1. 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

A

b. Polymerizes to form tactoid
crystals

47
Q
  1. 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

A

d. Decreased production of a globin
chain

48
Q
  1. 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

A

d. Hemoglobin electrophoresis at
acid pH

49
Q
  1. Megaloblastic episodes in SCD can be
    prevented by prophy-lactic administration of:

a. Iron
b. Folic acid
c. Steroids
d. Erythropoietin

A

b. Folic acid

49
Q
  1. 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

A

c. Higher levels of Hb F are present

50
Q
  1. Painful crises in patients with SCD occur as a result of:

a. Splenic sequestration
b. Aplasia
c. Vasoocclusion
d. Anemia

A

c. Vasoocclusion

50
Q
  1. A patient presents with mild normochromic, normocytic anemia. On the peripheral blood film, there are a few target cells, rare nucleated RBCs, and hexagonal crystals within and lying outside of the RBCs. Which abnormality in the hemoglobin molecule is most likely?

a. Decreased production of b chains
b. Substitution of lysine for glutamic
acid at position 6 of the b chain
c. Substitution of tyrosine for the
proximal histidine in the b chain
d. Double amino acid substitution in the
b chain

A

b. Substitution of lysine for glutamic
acid at position 6 of the b chain

51
Q
  1. A well-mixed specimen obtained for a CBC has a brown color. The patient is being
    treated with a sulfonamide for a bladder
    infection. Which of the following could explain the brown color?

a. The patient has Hb M.
b. The patient is a compound
heterozygote for Hb S and thalassemia.
c. The incorrect anticoagulant was used.
d. Levels of Hb F are high.

A

a. The patient has Hb M.

52
Q
  1. Through routine screening, prospective
    parents discover that they are both
    heterozygous for Hb S. What percentage of
    their children potentially could have sickle cell anemia (Hb SS)?

a. 0%
b. 25%
c. 50%
d. 100%

A

b. 25%

53
Q
  1. The screening test for Hb S that uses a
    reducing agent, such as sodium dithionite, is based on the fact that hemoglobins that
    sickle:

a. Are insoluble in reduced,
deoxygenated form
b. Form methemoglobin more readily
and cause a color change
c. Are unstable and precipitate as Heinz
bodies
d. Oxidize quickly and cause turbidity

A

a. Are insoluble in reduced,
deoxygenated form

54
Q
  1. DNA analysis documents a patient has
    inherited the sickle mutation in both b-globin genes. The two terms that best describe this genotype are:

a. Homozygous/trait
b. Homozygous/disease
c. Heterozygous/trait
d. Heterozygous/disease

A

b. Homozygous/disease

55
Q
  1. In which of the following geographic areas is Hb S most prevalent?

a. India
b. South Africa
c. United States
d. Sub-Saharan Africa

A

d. Sub-Saharan Africa

56
Q
  1. Which hemoglobinopathy is more common in Southeast Asian patients?

a. Hb S
b. Hb C
c. Hb O
d. Hb E

A

d. Hb E

57
Q
  1. Which of the following Hb S compound
    heterozygotes exhibits the mildest symptoms?

a. Hb S-b-thalassemia
b. Hb SG
c. Hb SC-Harlem
d. Hb SC

A

b. Hb SG

58
Q
  1. A 1-year-old Indian patient presents with anemia, and both parents claim to have an “inherited anemia” but can’t remember the type. The peripheral blood shows target cells, and the hemoglobin solubility is negative. Alkaline hemoglobin
    electrophoresis shows a single band at
    the “Hb C” position and a small band at
    the “Hb F” position. Acid hemoglobin
    electrophoresis shows two bands. The
    most likely diagnosis is:

a. Hb CC
b. Hb AC
c. Hb CO
d. Hb SC

A

c. Hb CO

59
Q
  1. Unstable hemoglobins exhibit all of the
    following findings except:

a. Globin chains that precipitate
intracellularly
b. Heinz body formation
c. Elevated reticulocyte count
d. Only homozygotes are
symptomatic

A

d. Only homozygotes are
symptomatic

60
Q
  1. Thalassemia is caused by:

a. Structurally abnormal hemoglobins
b. Absent or reduced synthesis of
a polypeptide chain of hemoglobin
c. Excessive absorption of iron
d. Reduced or absent protoporphyrin
synthesis

A

b. Absent or reduced synthesis of
a polypeptide chain of hemoglobin

61
Q
  1. Thalassemia is more prevalent in
    individuals from areas along the tropics
    because it confers:

a. Resistance to heat in
heterozygotes with a thalassemia
mutation
b. Selective advantage against
tuberculosis
c. Resistance to severe malaria in
heterozygotes with a thalassemia
mutation
d. Selected advantage against
tick-borne illnesses

A

c. Resistance to severe malaria in
heterozygotes with a thalassemia
mutation

62
Q
  1. The hemolytic anemia and ineffective
    erythropoiesis associated with b-thalassemia is due to:

a. A structurally abnormal hemoglobin
b. Oxidation of hemoglobin to Heinz
bodies
c. Uncoupling of the RBC membrane
from the cytoskeleton
d. Precipitation of excess a chains
in RBCs and their precursors

A

d. Precipitation of excess a chains
in RBCs and their precursors

63
Q
  1. b-thalassemia minor (heterozygous) usually exhibits:

a. Increased Hb H
b. 10% to 35% Hb F
c. No Hb A
d. Increased Hb A2

A

d. Increased Hb A2

64
Q
  1. RBC morphologic features in
    b-thalassemia major usually include:

a. Microcytes, hypochromia, target
cells, RBC inclusions, NRBCs
b. Macrocytes, acanthocytes, target
cells, polychromasia, NRBCs
c. Microcytes, hypochromia, target
cells, sickle cells, elliptocytes
d. Macrocytes, hypochromia, target
cells, RBC inclusions, NRBCs

A

a. Microcytes, hypochromia, target
cells, RBC inclusions, NRBCs

65
Q
  1. b-thalassemia major of the genotype
    b0/b0 can be differentiated from the b1/b1
    genotype by the amount of:

a. Hb A
b. Hb A2
c. Hb F
d. Hb H

A

a. Hb A

66
Q
  1. Homozygotes for deletional-type HPFH are characterized by:

a. 10% to 35% Hb F with normal
RBC morphology
b. 100% Hb F with slightly
hypochromic, microcytic RBCs
c. 1% Hb F with normal RBC
morphology
d. 5% to 15% Hb F with slightly
hypochromic, microcytic RBCs

A

b. 100% Hb F with slightly
hypochromic, microcytic RBCs

67
Q
  1. What abnormal hemoglobin is present in
    adults with a-thalassemia, genotype (– –/a
    –)?

a. A2
b. F
c. H
d. Bart

A

c. H

68
Q
  1. When one a gene is deleted (a–/aa), a
    patient has:

a. Normal hemoglobin levels
b. Mild anemia (hemoglobin range 9 to
11 g/dL)
c. Moderate anemia (hemoglobin range
7 to 9 g/dL)
d. Marked anemia requiring regular
transfusions

A

a. Normal hemoglobin levels

69
Q
  1. Hb Bart is composed of:

a. Two a and two b chains
b. Two e and two g chains
c. Four b chains
d. Four g chains

A

d. Four g chains

70
Q
  1. In which part of the world is the a gene
    mutation causing Hb Bart hydrops fetalis (–
    –/– –) most common?

a. Northern Africa
b. Mediterranean
c. Middle East
d. Southeast Asia

A

d. Southeast Asia

71
Q
  1. A patient with a hemoglobin concentration of 8.0 g/dL and an MCV of 62 fL had microcytes, target cells, and a few sickle cells on his peripheral blood film. High-performance liquid chromatography showed 25% Hb A, 65% Hb S, 6% Hb A2, and 4% Hb F. These results are most compatible with:

a. Sickle cell trait
b. Sickle cell anemia
c. Hb S-b0-thalassemia
d. Hb S-b+-thalassemia

A

d. Hb S-b+-thalassemia

72
Q
  1. Hb H inclusions in a supravital stain
    preparation appear as:

a. A few large, blue, round bodies in the
RBCs with aggre-gated reticulum
b. Uniformly stained blue cytoplasm in
the RBC
c. Small, evenly distributed,
greenish-blue granules that pit the
surface of RBCs
d. Uniform round bodies that adhere to
the inner RBC membrane

A

c. Small, evenly distributed,
greenish-blue granules that pit the
surface of RBCs

73
Q
  1. A 9-month-old infant of Asian heritage is seen for severe fatigue and pallor. Her
    hemoglobin concentration is 6.5 g/dL with an MCV of 59 fL; microcytosis, hypochromia, poikilocy-tosis, basophilic stippling, Howell Jolly bodies, Pappenheimer bodies, and nucleated
    RBCs are noted on the peripheral blood film. High-performance liquid chromatography showed 0% Hb A, 96% Hb F, and 4% Hb A2. These findings should lead the physician to suspect:

a. b-thalassemia major, b0/b0
b. b-thalassemia major, b1/b1
c. Severe iron deficiency anemia
d. Homozygous a-thalassemia (– –/– –)

A

a. b-thalassemia major, b0/b0

74
Q
  1. Which of the following laboratory findings is inconsistent with b-thalassemia minor?

a. A slightly elevated RBC count and
marked microcytosis
b. Target cells and basophilic stippling
on the peripheral blood film
c. Hemoglobin level of 10 to 13 g/dL
d. Elevated MCHC and spherocytic
RBCs

A

d. Elevated MCHC and spherocytic
RBCs

75
Q
  1. Which of the following inherited leukocyte disorders is caused by a mutation in the lamin B receptor?

a. Pelger-Huët anomaly
b. Chédiak-Higashi disease
c. Alder-Reilly anomaly
d. May-Hegglin anomaly

A

a. Pelger-Huët anomaly

76
Q
  1. Which of the following inherited leukocyte disorders involves mutations in nonmuscle myosin heavy-chain IIA?

a. Pelger-Huët anomaly
b. Chédiak-Higashi disease
c. Alder-Reilly anomaly
d. May-Hegglin anomaly

A

d. May-Hegglin anomaly

77
Q
  1. Which of the following inherited leukocyte disorders might be seen in Hurler syndrome?

a. Pelger-Huët anomaly
b. Chédiak-Higashi disease
c. Alder-Reilly anomaly
d. May-Hegglin anomaly

A

c. Alder-Reilly anomaly

78
Q
  1. Which of the following lysosomal storage
    diseases is characterized by macrophages
    with striated cytoplasm and storage of
    glucocerebroside?

a. Sanfilippo syndrome
b. Gaucher disease
c. Fabry disease
d. Niemann-Pick disease

A

b. Gaucher disease

79
Q
  1. The neutrophils in chronic granulomatous disease are inca-pable of producing:

a. Hydrogen peroxide
b. Hypochlorite
c. Superoxide
d. All of the above

A

d. All of the above

80
Q
  1. Individuals with X-linked SCID have a
    mutation that affects their ability to synthesize:

a. Deaminase
b. Oxidase
c. IL-2 receptor
d. IL-8 receptor

A

c. IL-2 receptor

81
Q
  1. An absolute lymphocytosis with reactive
    lymphocytes sug-gests which of the following conditions?

a. DiGeorge syndrome
b. Bacterial infection
c. Parasitic infection
d. Viral infection

A

d. Viral infection

82
Q
  1. What leukocyte cytoplasmic inclusion is
    composed of ribosomal RNA?

a. Primary granules
b. Toxic granules
c. Döhle bodies
d. Howell-Jolly bodies

A

c. Döhle bodies

83
Q
  1. The expected complete blood cell count
    (CBC) results for women in active labor would include:

a. High total white blood cell (WBC)
count with increased lymphocytes
b. High total WBC count with a slight
shift to the left in neutrophils
c. Normal WBC count with increased
eosinophils
d. Low WBC count with increased
monocytes

A

b. High total WBC count with a slight
shift to the left in neutrophils

84
Q
  1. Which of the following is true of an absolute increase in lymphocytes with reactive morphology?

a. The population of lymphocytes
appears morphologi-cally
homogeneous.
b. They are usually effector B cells.
c. The reactive lymphocytes have
increased cytoplasm with variable
basophilia.
d. They are most commonly seen in
bacterial infections.

A

c. The reactive lymphocytes have
increased cytoplasm with variable
basophilia.

85
Q
  1. Lymphomas differ from leukemias in that they are:

a. Solid tumors
b. Not considered systemic diseases
c. Never found in peripheral blood
d. Do not originate from hematopoietic
cells

A

a. Solid tumors

86
Q
  1. Which one of the following viruses is known to cause lymphoid neoplasms in humans?

a. HIV-1
b. HTLV-1
c. Hepatitis B
d. Parvovirus B

A

b. HTLV-1

87
Q
  1. Loss-of-function of tumor suppressor genes increase the risk of hematologic neoplasms by:

a. Suppressing cell division
b. Activating tyrosine kinases which
promote proliferation
c. Promoting excessive apoptosis of
hematopoietic cells
d. Allowing cells with damaged DNA
to progress through the cell cycle

A

d. Allowing cells with damaged DNA
to progress through the cell cycle

88
Q
  1. Oncogenes are said to act in a dominant
    fashion because:

a. Leukemia is a dominating disease
that is systemic
b. The oncogene product is a
gain-of-function mutation
c. A mutation in only one allele is
sufficient to promote a malignant
phenotype
d. They are inherited by autosomal
dominant transmission

A

c. A mutation in only one allele is
sufficient to promote a malignant
phenotype

HEMATOLOGY-MTAP (10 decks)

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