CHAPTER 19 Flashcards by Prince Mella

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

c. Fatigue, infection, and bleeding

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Idiopathic acquired aplastic anemia is due to a(n):

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

d. Unknown cause

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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

b. Destruction of stem cells by
autoimmune T cells

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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

d. Very severe

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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

b. Macrocytosis, thrombocytopenia, and
neutropenia

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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

d. Bone marrow transplant with an
HLA-identical sibling

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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

c. Diepoxybutane-induced chromosome
breakage

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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

d. Premature death of hematopoietic
stem cells

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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

c. Only erythropoiesis is affected

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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

d. Congenital dyserythropoietic anemia

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RBC destruction that occurs when macrophages ingest and destroy RBCs is termed:

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

b. Macrophage mediated

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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

d. Elevated urinary urobilinogen level

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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. Inadequate production of
erythropoietin

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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. Reticulocytosis

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

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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

b. Yellow skin and eyes

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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.

c. Evidence of increased protoporphyrin
catabolism is lacking.

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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

c. Reticulocyte count

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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. Acquired, fragmentation

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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

d. Spherocytes on the peripheral blood
film

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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

c. Macrophage-secreted indirect bilirubin

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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

c. Decreased Positive Positive

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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

c. Increased osmotic fragility, negative
DAT result

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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

c. Increased MCHC

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2. 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

b. A mutated RBC membrane protein affecting vertical protein interactions

4. 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. Defects in horizontal membrane protein interactions

5. 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

b. Elliptical RBCs

6. 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. Increased MCV and normal RDW

7. Acanthocytes are found in association with: a. Abetalipoproteinemia b. G6PD deficiency c. Rh deficiency syndrome d. Vitamin B12 deficiency

a. Abetalipoproteinemia

8. 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. Acute hemolytic anemia caused by drug exposure or infections

9. 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

c. False increase in enzyme activity due to reticulocytosis

10. 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

d. Pyruvate kinase deficiency

11. 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

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

12. 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

c. Hypoplastic PNH

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

c. Intravascular RBC fragmentation

2. 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. Schistocytosis and thrombocytopenia

3. 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

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

4. 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

b. Prothrombin time and partial thromboplastin time

5. 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

c. Decreased serum haptoglobin

6. 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

c. P. knowlesi

8. 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

d. Schistocytes and microspherocytes

7. 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

c. Babesiosis

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

b. Allo- or autoantibody against an RBC antigen

9. 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

c. TTP

10. 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. Aspartate aminotransferase

4. 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

d. Demonstration of IgG a

3. 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. Spherocytes

2. The pathophysiology of immune hemolysis with IgM antibodies always involves: a. Complement b. Autoantibodies c. Abnormal hemoglobin molecules d. Alloantibodies

a. Complement

5. 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

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

6. 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

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

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

8. 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

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

10. 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

c. RBC autoantibody induction

9. Chronic secondary CAD is most often associated with: a. Antibiotic therapy b. M. pneumoniae infection c. B cell malignancies d. Infectious mononucleosis

c. B cell malignancies

11. 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

c. The mother was previously immunized to the D antigen

2. 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

b. Polymerizes to form tactoid crystals

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

d. Decreased production of a globin chain

5. 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

d. Hemoglobin electrophoresis at acid pH

4. Megaloblastic episodes in SCD can be prevented by prophy-lactic administration of: a. Iron b. Folic acid c. Steroids d. Erythropoietin

b. Folic acid

3. 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

c. Higher levels of Hb F are present

9. Painful crises in patients with SCD occur as a result of: a. Splenic sequestration b. Aplasia c. Vasoocclusion d. Anemia

c. Vasoocclusion

6. 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

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

7. 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. The patient has Hb M.

8. 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%

b. 25%

10. 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. Are insoluble in reduced, deoxygenated form

11. 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

b. Homozygous/disease

12. In which of the following geographic areas is Hb S most prevalent? a. India b. South Africa c. United States d. Sub-Saharan Africa

d. Sub-Saharan Africa

13. Which hemoglobinopathy is more common in Southeast Asian patients? a. Hb S b. Hb C c. Hb O d. Hb E

d. Hb E

14. 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

b. Hb SG

15. 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

c. Hb CO

16. 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

d. Only homozygotes are symptomatic

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

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

2. 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

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

3. 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

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

4. b-thalassemia minor (heterozygous) usually exhibits: a. Increased Hb H b. 10% to 35% Hb F c. No Hb A d. Increased Hb A2

d. Increased Hb A2

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

6. 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. Hb A

7. 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

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

8. What abnormal hemoglobin is present in adults with a-thalassemia, genotype (– –/a –)? a. A2 b. F c. H d. Bart

c. H

10. 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. Normal hemoglobin levels

9. 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

d. Four g chains

11. 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

d. Southeast Asia

12. 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

d. Hb S-b+-thalassemia

13. 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

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

15. 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. b-thalassemia major, b0/b0

14. 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

d. Elevated MCHC and spherocytic RBCs

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. Pelger-Huët anomaly

2. 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

d. May-Hegglin anomaly

3. 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

c. Alder-Reilly anomaly

4. 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

b. Gaucher disease

5. The neutrophils in chronic granulomatous disease are inca-pable of producing: a. Hydrogen peroxide b. Hypochlorite c. Superoxide d. All of the above

d. All of the above

6. 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

c. IL-2 receptor

7. 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

d. Viral infection

8. What leukocyte cytoplasmic inclusion is composed of ribosomal RNA? a. Primary granules b. Toxic granules c. Döhle bodies d. Howell-Jolly bodies

c. Döhle bodies

9. 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

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

10. 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.

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

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. Solid tumors

2. 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

b. HTLV-1

3. 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

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

4. 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

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