ASCP MLT BOC- Study Questions- Hematology

A physician wants to obtain a measure of a patient's

iron stores. Which of the following tests would be the

most suitable?

a. Serum iron

b. Serum transferrin (TIBC)

c. Serum ferritin

d. Transferrin saturation

c. Serum ferritin concentrations reflect the body's

storage of ferritin. Serum iron measures free iron, and serum transferrin provides a measure of how

many binding sites are available to bind iron and is

used with transferrin saturation, the percentage of

sites available to carry iron.

A 68-year-old woman visited her physician with

reports of fatigue and weakness. A CBC was ordered,

and the patient's results were as follows:

RBC 2.50 1012/L Hct 18.8% MCH 24.8 pg

Hgb 6.2 g/dL MCV 75.2 fL MCHC 33%

Which of the following would be a plausible diagnosis

for this patient?

a. Iron-deficiency anemia

b. Vitamin B12 deficiency

c. Anemia of chronic inflammation

d. Hemochromatosis

2. a. Iron-deficiency anemia resulting from the

decreased mean corpuscular volume (MCV) and

mean corpuscular hemoglobin (MCH), indicating

hypochromic microcytic cells. Vitamin B12 deficiency

typically exhibits macrocytosis. Anemia

of chronic inflammation tends to exhibit a mild

anemia, with a hemoglobin value of approximately

9 to 11 g/dL. Hemochromatosis is not an

anemia and, untreated, exhibits a normal to elevated

red blood cell count.

3. A peripheral smear shows a decreased RBC count

with microcytic, hypochromic cells with small grapelike

inclusions in the RBCs on bothWright stain and

Prussian blue stain. This is consistent with:

a. Iron-deficiency anemia

b. Sideroblastic anemia

c. Pernicious anemia

d. b-Thalassemia minor

3. b. The inclusions suggest the presence of excess

storage iron. Sideroblastic anemia is a disorder

characterized by elevated iron stores resulting

from an inability to incorporate iron into heme.

The inclusions suggest the presence of excess

storage iron. Ringed sideroblasts may also be

found in bone marrow examinations. Irondeficiency

anemia shows a microcytic hypochromic

anemia; however, it is characterized by a lack

of iron. Pernicious anemia is a megaloblastic anemia.

Thalassemia minor shows a microcytic hypochromic

anemia; however, it often has an elevated

red blood cell count with hypochromic, microcytic

cells but would not normally show iron

inclusions (although transfusion-dependent thalassemias

may exhibit transfusion-associated

iron excess.

4. Given the following results of iron studies, which disorder

is the most likely?

# Serum iron ↑ TIBC

# Ferritin # % Saturation

a. Iron-deficiency anemia

b. Sideroblastic anemia

c. Anemia of chronic inflammation

d. Hemochromatosis

a. Iron-deficiency anemia is most likely, because

sideroblastic anemia and hemochromatosis show

increased iron and ferritin with a decreased total

iron-binding capacity (TIBC) whereas anemia of

chronic inflammation has decreased serum iron

and percent saturation but normal-to-increased

serum ferritin.

5. Acquired sideroblastic anemia may be present in all

of the following except:

a. Alcoholism

b. Lead poisoning

c. Malabsorption

d. Myelodysplastic syndromes

c. Gastrointestinal disease may lead to malabsorption,

which could possibly affect iron absorption.

Alcoholism and lead poisoning can lead to a secondary

sideroblastic anemia, and primary sideroblastic

anemia may be seen in myelodysplastic

syndromes, such as refractory anemia with ringed

sideroblasts (RARS).

A patient has a macrocytic anemia, and the physician

suspects pernicious anemia. Which test would best

rule in a definitive diagnosis of pernicious anemia?

a. Homocysteine

b. Intrinsic factor antibodies

c. Ova and parasite examination for D. latum

d. Bone marrow examination

b. Intrinsic factor antibodies would be present in

patients with a true megaloblastic anemia, because

it is characterized by the destruction of parietal

cells, which produce the intrinsic factor needed

for B12 absorption. Homocysteine is elevated in

both vitamin B12 and folic acid deficiencies.

Diphyllobothrium latum can cause megaloblastic anemia because it competes for vitamin B12 in the

intestines; however, testing for ova and parasites

alone will not define the diagnosis. A bone marrow

examination could determine that megaloblastic

features were present; however, it would not be

specific for pernicious anemia.

Megaloblastic anemias result from which of the

following?

a. Deficiencies in free erythrocyte protoporphyrin

b. Deficiencies in Vitamin B12 and folic acid

c. Increases in iron and hepcidin

d. Decreases in liver function

b. Megaloblastic anemias result from deficiencies in

vitamin B12 and folic acid. Both are needed for

normal cell maturation. Iron and hepcidin play

a role in anemias with iron problems, whereas

decreased free erythrocyte protoporphyrin (FEP)

is seen in some porphyrias. Decreased liver function,

alcoholism, and severe hypothyroidism can

cause macrocytic anemia, but the anemia is not

megaloblastic.

A patient's bone marrow showed erythroid hyperplasia

with signs of dysplastic maturation, particularly

in the RBC precursors. This is consistent with which

of the following?

a. Sickle cell anemia

b. b-Thalassemia major

c. Pernicious anemia

d. G6PD deficiency

c. Pernicious anemia is a megaloblastic anemia that

results from defective DNA synthesis from lack of

vitamin B12, often showing dysplastic changes in

the cells and sometimes requiring a bone marrow

examination to confirm the deficiency, particularly

to differentiate from myelodysplastic syndromes.

The others do not have a need for bone

marrow examination.

The CBC for a 57-year-old man had the

following results. Which tests would be best to

order next?

RBC 2.50 1012/L

Hct 26.0%

MCH 34 pg

Hgb 8.5 g/dL

MCV 104 fL

MCHC 33%

a. Iron studies

b. Vitamin B12 and folic acid levels

c. Bone marrow examination

d. Intrinsic factor antibodies

b. Vitamin B12 and folic acid are the best place to

start in further investigating this patient's anemia,

because this will determine the specific

follow-up most valuable to the physician. Iron

studies could be performed, but a deficiency is

unlikely given the macrocytic appearance of the

red blood cells. Bone marrow examination is

not usually performed unless confirmation of

other testing or rule out of myelodysplastic syndrome

(MDS) is needed. Intrinsic factor antibody

assays may be used to further work up the case if

vitamin B12 levels are decreased, because the reason

for the decrease would need to be confirmed

or ruled out.

The majority of acquired aplastic anemia cases usually

results from which of the following?

a. Unknown causes

b. Pregnancy

c. Chloramphenicol exposure

d. Radiation exposure

a. Approximately 70% of acquired aplastic anemia

cases are idiopathic (Rodak, 2012). It can occur as

a result of other stimuli, including various drugs,

radiation exposure, and viral infections.

11. Which of the following values is the most likely to be

normal in a patient with aplastic anemia?

a. RBC count

b. Absolute neutrophil count

c. Absolute lymphocyte count

d. Platelet count

c. The absolute lymphocyte count is most likely to

be normal, because lymphocytes may also reside

in lymphoid tissue beyond the bone marrow.

The others and their precursors are primarily

found in the bone marrow and tend to have a

shorter life span in circulation.

12. Fanconi's anemia is an inherited aplastic anemia with

mutations that lead to:

a. Increased chromosome fragility

b. Myelophthisic anemia

c. Pancreatic issues

d. RBC enzymatic defects

a. Fanconi's anemia is characterized by mutations in

a group of genes that lead to fragile chromosomes,

which break easily and may not be able to repair

themselves.

13. Which of the following is decreased in cases of intravascular

hemolytic anemia?

a. Bilirubin

b. Urine hemosiderin

c. Haptoglobin

d. Plasma hemoglobin

13. c. Haptoglobin is a protein that picks up free hemoglobin,

and it frequently decreases as it is used up

when free hemoglobin (Hgb) is present in excess

of haptoglobin's carrying capacity. Bilirubin, both

total and unconjugated, is increased with the

increased red blood cell destruction. Plasma

Hgb and urine hemosiderin are also increased

because of the excesses of free Hgb.

Typical CBC findings in hemolytic anemia include:

a. Microcytic, hypochromic cells with increased

poikilocytosis

b. Macrocytic, normochromic cells with increased

polychromasia

c. Microcytic, normochromic cells with increased

poikilocytosis

d. Macrocytic, hypochromic cells with increased

polychromasia

14. b. Macrocytic, normochromic cells with increased

polychromasia are present in most cases of hemolytic

anemia, because reticulocytes are being

released prematurely from the bone marrow to

replace cells being destroyed. Microcytic and

hypochromic cells are usually seen in disorders

of iron/heme metabolism.

15. Which of the following disorders does not have a

hemolytic component?

a. Sickle cell anemia

b. Autoimmune hemolytic anemia

c. Glucose-6-phosphate dehydrogenase deficiency

d. Anemia of chronic disease

d. Anemia of chronic disease is characterized by a

block in iron incorporation and is a mild anemia,

not characterized by increased cell destruction.

Sickle cell, active glucose-6-dehydrogenase

(G6PD) deficiency, and autoimmune hemolytic

anemia are types of hemolytic anemia.

16. A patient presents with evidence of a hemolytic

anemia. Spherocytes, polychromasia, and macrocytosis

are observed. Which of the following

would best help to distinguish the cause of the

anemia?

a. Osmotic fragility

b. DAT

c. G6PD activity assay

d. Vitamin B12 level

b. The direct antiglobulin test (DAT) would be the

best test to begin determining the cause of anemia,

because it can help determine if spherocytes are

the result of immune activity or if they are due

to abnormal red blood cell skeletal protein

interactions. Osmotic fragility will be decreased

in the presence of spherocytes. Glucose-6-

dehydrogenase (G6PD) activity would be useful

only if G6PD deficiency was present. Vitamin

B12 is used to determine the cause of macrocytic

anemia and does not usually result in spherocytes.

17. Paroxysmal nocturnal hemoglobinuria is characterized

by flow cytometry results that are:

a. Negative for CD55 and CD59

b. Positive for CD55 and CD59

c. Negative for CD4 and CD8

d. Positive for all normal CD markers

a. The PIGA gene codes for glycophosphatidylinositol

(GPI)-anchored proteins. Paroxysmal nocturnal

hemoglobinuria shows a mutation in the PIGA

gene, which results in deficiencies in GPI proteins,

indicated by a negative CD55 and CD59.

18. G6PD deficiency episodes are related to which of the

following?

a. Exposure to oxidant damage

b. Defective globin chains

c. Antibodies to RBCs

d. Abnormal protein structures

a. Glucose-6-dehydrogenase (G6PD)deficiency shows

lack of enzyme activity that is needed for the reduction

of glutathione, whichin turnworks to deal with

protecting hemoglobin from oxidant damage.

Defective globin chains can be seen in hemolytic

hemoglobinopathies or thalassemia. Antibodies to

red blood cells are present in immune-mediated

hemolytic anemias, and abnormal protein structure

is seen in disorders such as hereditary elliptocytosis

or hereditary spherocytosis.

19. Which of the following disorders is not classified as a

microangiopathic hemolytic anemia?

a. Disseminated intravascular coagulation

b. Hemolytic uremic syndrome

c. Traumatic cardiac hemolytic anemia

d. Thrombotic thrombocytopenic purpura

c. All are microangiopathic hemolytic anemias, with

the exception of traumatic cardiac hemolytic anemia,

because they feature intravascular hemolysis

resulting from red blood cells (RBCs) shearing when they contact microclots in the circulation.

Traumatic cardiac hemolytic anemia is macroangiopathic,

because the hemolysis occurs when

RBCs move through implanted cardiac devices

or patients with cardiac valve issues.

A previously healthy 36-year-old woman with

visited her physician because of a sudden onset of easy

bruising and bleeding. Of the following, which is the

most likely cause of her laboratory results?

WBC 10.5 109/L

RBC 3.00 1012/L

Hgb 8.0 g/dL

Hct 25.0%

MCV 83 fL

MCH 26 pg

MCHC 32%

Platelets 18 109/L

Differential:

Normal WBCs with moderate

schistocytes and polychromasia

PT: 12.8 seconds

aPTT: 34 seconds

a. Sickle cell anemia

b. Chronic myelogenous leukemia

c. Disseminated intravascular coagulation

d. Thrombotic thrombocytopenic purpura

d. Thrombotic thrombocytopenic purpura (TTP) is

the most likely cause of the laboratory results,

because it is consistent with the anemia and

thrombocytopenia with the presence of schistocytes.

The patient is exhibiting normal coagulation

results, which would be increased in

disseminated intravascular coagulation (DIC).

Chronic myelogenous leukemia could show

decreased red blood cell and platelet count;

however, this is a younger patient with normal

white blood cells. Sickle cell disease is unlikely

in a previously healthy female, and the decreased

platelets and schistocytes point more to a microangiopathic

hemolytic anemia (MAHA).

21. Warm autoimmune hemolytic anemia is usually

caused by which of the following?

a. IgA antibodies

b. IgG antibodies

c. IgM antibodies

d. Complement

b. The majority of warm autoimmune hemolytic

anemia cases involve IgG antibodies, although

other antibodies, such as IgA or IgM, may be

implicated in rare cases.

22. Which of the following conditions is not associated

with secondarywarmautoimmune hemolytic anemia?

a. CLL

b. Idiopathic onset

c. Rheumatoid arthritis

d. Viral infections

22. b. Idiopathic onset is an unknown cause of warm

autoimmune hemolytic anemia (WAIHA). SecondaryWAIHA

is usually associated with chronic

lymphoid disorders, viral infections, and autoimmune

disorders.

23. The mutation seen in sickle cell anemia is:

a. b6Glu!Val

b. b6Glu!Lys

c. b26Glu!Lys

d. b63Glu!Arg

a. b6Glu ! Val is the mutation seen in sickle cell anemia.

b6Glu ! Lys is the mutation seen in hemoglobin

(Hgb) C, b26Glu ! Lys is the mutation seen in

Hgb E, and b63Glu ! Arg is seen in Hgb Zurich.

24. The majority of hospitalizations associated with

sickle cell anemia are due to:

a. Cardiomegaly

b. Cholelithiasis

c. Pneumonia

d. Vasoocclusion

d. All of these conditions are associated with sickle

cell disease; however, vasoocclusion is common

and leads to painful crises that often result in hospital

visits.

25. Patients with sickle cell trait usually have RBC morphology

that includes which of the following?

a. Normocytic, normochromic RBCs with occasional

target cells

b. Normocytic, normochromic RBCs with rare

sickle cells

c. Hypochromic, microcytic RBCs with moderate

target cells

d. Macrocytic, normochromic cells with

occasional NRBCs

a. Patients with sickle cell trait usually have no clinical

symptoms or abnormalities on their complete

blood count, although they may exhibit occasional

target cells. Under extreme stress or hypoxia,

patients may have serious complications

similar to those seen in actual sickle cell disease.

26. Which laboratory test is best used for definitive diagnosis

of sickle cell anemia?

a. Solubility testing

b. Hemoglobin electrophoresis

c. Peripheral smear review for sickle cells

d. Bone marrow analysis

b. Hemoglobin (Hgb) electrophoresis, highperformance

liquid chromatography, or isoelectric

focusing is the best means for determining the specific

Hgbs present in a patient sample. Solubility

testing is a good screen to look for abnormal Hgbs,

but does not determine specific Hgb presence or

approximate quantities. Bone marrow analysis is

an invasive technique and does not provide a definitive listing for the Hgbs present. Peripheral

smear review may showthe presence of sickle cells;

however, it will not determine if Hgb S is present in

the case of someone heterozygous for Hgb S.

27. A peripheral smear review shows mildly anemic sample

with target cells and oblong hexagonal crystalloids.

What is a possible identity for the crystalloids?

a. Hemoglobin S

b. Hemoglobin C

c. Hemoglobin SC

d. Hemoglobin E

27. b. The crystals are likely hemoglobin (Hgb) C,

because this abnormal Hgb tends to polymerize

in short hexagonal crystals. Hgb S will polymerize

into sickle cells. Hgb SC polymerizes into forms

that are a hybrid of Hgb S and Hgb C that look

like fingers or birds. Hgb E does not form specific

crystalloid shapes.

An 18-year-old man has a CBC done when visiting

his physician for a persistent sore throat. He has

the following results:

WBC 12.5 109/L

RBC 6.00 1012/L

Hgb 10.0 g/dL

Hct 30.0%

MCV 60 fL

MCH 20 pg

MCHC 33%

Platelet 218 109/L

Which of the following is most likely?

a. This patient is normal with a slightly elevated

WBC count because of his sore throat

b. This patient has infectious mononucleosis and

warm autoimmune hemolytic anemia

c. This patient is likely to have b-thalassemia

minor

d. There is a specimen quality issue because of a cold

agglutinin

28. c. This patient most likely has thalassemia minor, in

addition to his sore throat. The patient has an elevated

red blood cell (RBC) count with a disproportionately

low hemoglobin and hematocrit,

which is often seen in thalassemia minor. Warm

autoimmune hemolytic anemia would lead to a

decrease in the RBC count. Results are not consistent

with a cold agglutinin.

29. Hemoglobin H disease is described as:

a. / a

b. a/ a

c. /bb

d. b/ b

a. Hemoglobin H is the result of a three-gene deletion

on the a gene. A two-gene deletion would

result in a-thalassemia minor.

30. A 3-year-old female patient is seen in the hematology

clinic to investigate the cause of her persistent anemia.

Hemoglobin electrophoresis was ordered, and

results showed an elevation in Hgb F, with a small

increase in Hgb A2. What is the most likely disorder

based on these results?

a. a-Thalassemia major

b. b-Thalassemia major

c. a-Thalassemia minor

d. Hemoglobin H disease

30. b. This is likely a case of b-thalassemia because

hemoglobin (Hgb F) (a2g2) and Hgb A2 (a2d2)

are increased. Thus a chains are able to be produced;

however, b chains are lacking so no Hgb

A (a2b2) is present. The other disorders listed

are all covered in the a-thalassemia group.

31. A 36-year-old male patient has a CBC performed as

part of a routine work physical. The WBC count was

6.5 109/L with a differential count of 48% neutrophils,

40% lymphocytes, 8% monocytes, 3% eosinophils,

and 1% basophils. The majority of the

neutrophils were mature but hyposegmented, showing

bandlike or single nuclei. What disorder would be

suspected?

a. Alder-Reilly anomaly

b. Leukocyte adhesion deficiency

c. Pelger-Huet anomaly

d. Reed Sternberg syndrome

c. This is consistent with Pelger-Huet anomaly, which

is characterized by mature neutrophils, but hyposegmentation

in the majority of neutrophils.

Alder-Reilly anomaly features abnormal granulation,

leukocyte adhesion deficiency shows relatively

normal looking but functionally abnormal

neutrophils.

A 38-year-old male patient has the following CBC

results:

WBC 32.5 109/L

RBC 5.50 1012/L

Hgb 16.0 g/dL

Hct 48.0%

Platelet 225 109/L

Differential:

49% segmented neutrophils,

9% bands,

25% lymphocytes,

9% monocytes,

1% eosinophils,

4% metamyelocytes,

3% myelocytes;

RBC and

platelet morphology

appear normal

Which of the following conditions is the most likely

cause of these results?

a. Bacterial infection

b. CML

c. Refractory anemia

d. Viral infection

32. a. Acute bacterial infection is the most likely cause

of these results, with an elevated white blood cell

count and shift to the left. Although some similarities

may exist in the complete blood count picture

for chronic myelogenous leukemia (CML), the

patient is relatively young for the diagnosis. Additionally,

the patient appears to have normal red

blood cell (RBC) and platelet counts, which

may decrease with the neoplastic clone in CML.

Viral infections usually show elevations in lymphocyte

numbers. Refractory anemia is unlikely

because the patient has normal RBC counts.

33. Which of the following cytochemical stains is best

used to distinguish cells of monocytic origin?

a. a-Naphthyl acetate esterase

b. Naphthol AS-D chloroacetate esterase

c. Myeloperoxidase

d. Periodic acid-Schiff

a. a-Naphthyl acetate esterase can be used to exhibit

positive esterase activity in monocytes, whereas

neutrophils and lymphocytes usually stain negative. Naphthyl AS-D chloroacetate esterase

shows positive activity in granulocytic cells

with negative or weak reactions in monocytes.

Periodic acid-Schiff stains glycogen and mucoproteins,

and staining patterns may be used

to help in identification of various cell types.

Myeloperoxidase activity is strong in neutrophils

from the promyelocyte stage through maturity;

however, activity in monocytes is negative

or weak.

34. A positive tartrate-resistant acid phosphatase

(TRAP) stain is indicative of:

a. Burkitt's lymphoma

b. Chronic myelogenous leukemia

c. Hairy cell leukemia

d. Multiple myeloma

c. A positive tartrate-resistant acid phosphatase

(TRAP) stain is indicative of hairy cell leukemia,

because hairy cell lymphocytes produce large

amounts of acid phosphatase isoenzyme 5, which

is inhibited in the presence of tartaric acid. Most

other cells of various lines are positive for acid

phosphatase; however, they are not resistant to

the addition of tartaric acid because of normal

or decreased levels of isoenzyme 5.

35. Which mutation is shared by a large percentage of

patients with polycythemia vera, essential thrombocythemia,

and primary myelofibrosis?

a. BCR/ABL

b. JAK2 V617F

c. PDGFR

d. RUNX1

b. The JAK2 V617F mutation is present in numerous

cases of myeloproliferative neoplasms, including

polycythemia vera, essential thrombocythemia,

and primary myelofibrosis. BCR/ABL mutations

are seen in CML, and PDGFR mutations are seen

in neoplasms with eosinophilia. RUNX mutations

may be seen in some cases of acute leukemias.

36. A patient has a CBC and peripheral smear with an

elevated WBC count and left shift, suggestive of a

diagnosis of CML. Which of the following tests

would be the most helpful in confirming the suspected

diagnosis?

a. Cytochemical staining for myeloperoxidase

and LAP

b. Karyotyping for the Philadelphia chromosome

c. Flow cytometry for myeloid cell markers

d. Lymph node biopsies for metastasis

b. Although leukocyte alkaline phosphatase (LAP)

scores tend to be decreased in chronic myelogenous

leukemia (CML) and myeloid cells are present,

karyotyping for the presence of the Philadelphia

chromosome (9;22 translocation) is required for

the confirmation of a diagnosis of CML.

37. A patient has a splenomegaly, and his CBC shows a

left shift; bizarre RBCs, including dacryocytes; and

notable platelet abnormalities. Which of the following

would be the most helpful in determining the

patient's diagnosis?

a. Bone marrow biopsy

b. LAP staining

c. Karyotyping for the Philadelphia chromosome

d. Spleen biopsy

37. a. A bone marrow biopsy would help in confirming

a diagnosis of primary myelofibrosis, which is a

possible diagnosis suggested by the dacryocytes,

left shift, and abnormal platelets. If the patient

had primary myelofibrosis, the bone marrow

would likely show areas of fibrosis, in addition

to increases in megakaryocytes and abnormal

platelets. Splenomegaly would be explained by

extramedullary hematopoiesis, which is suggested

by the presence of dacryocytes. Leukocyte alkaline

phosphatase (LAP) staining and karyotyping

for the Philadelphia chromosome would be more

useful in determining a diagnosis of chronic myelogenous

leukemia.

38. Which of the following peripheral blood findings

would not be expected in a patient with a myelodysplastic

syndrome?

a. Hypogranular neutrophils

b. Binucleate neutrophils and NRBCs

c. Circulating micromegakaryocytes

d. Decreased vitamin B12 and folic acid

d. Myelodysplastic syndromes are characterized by

anemias refractory to normal treatment and

abnormal cellular appearance resulting from dyspoiesis

in the cell lines. Vitamin B12, folic acid,

and iron levels usually are normal; however, cells

do not mature normally.

39. The WHO system classifies this disorder as a Myeloproliferative/

Myelodysplastic syndrome.

a. Refractory Anemia with Ringed Sideroblasts

b. 5q Syndrome

c. Chronic Myelomonocytic Leukemia

d. Refractory Anemia with Multilineage Dysplasia

c. All of these disorders are classified as myelodysplastic

syndromes with the exception of chronic

myelomonocytic leukemia (CML), which was

moved into theWorld Health Organization classification

of myelodysplastic syndrome/myeloproliferative

neoplasms, along with juvenile

myelomonocytic leukemia, and atypical CML.

A 4-year-old male patient presents with a slightly elevated

WBC count, and occasional blasts are present

on the differential. Flow cytometry is performed with

the following results: CD10(+), CD19 (+), CD22(+),CD79a(+), TdT(+). Which of the following diagnoses

is the most likely?

a. Intermediate B-cell ALL

b. Pre-B-cell ALL

c. T-cell ALL

d. Pre-T-cell ALL

a. Intermediate B-cell acute lymphoblastic anemia

(ALL) is the most likely diagnosis because of the

B-cell markers (CD19 and CD22), in addition to

CD10, which are specifically seen in common

ALL (cALL), also known as intermediate B-cell

ALL. This is not a T-cell ALL, because no positive

T-cell markers are indicated in the results given.

41. Which of the following may predict a better prognosis

in patients with ALL?

a. The patient is a child

b. Peripheral blood blast counts greater than

30 109/L

c. The Philadelphia chromosome is present

d. The patient is hypodiploid

41. a. Prognosis is currently the best in children, as

opposed to in infants and adults. Elevated blast

counts or hypodiploidy are associated with a

poorer prognosis. The presence of the Philadelphia

chromosome has an unfavorable prognosis

in acute lymphoblastic anemia.

42. A 28-year-old female patient presented to the emergency

department with symptoms suggestive of

DIC. A CBC and coagulation studies were ordered.

The peripheral smear showed blasts and immature

cells with heavy granulation and Auer rods. Which

of the following disorders would be the most likely?

a. AML with t(9;11)(p22;q23); MLLT3-MLL

b. AML with t(15;17)(q22;q12); PML-RARa

c. ALL with t(12;21)(p13;q22); ETV6-RUNX1

d. ALL with t(9;22)(q34;q11.2); BCR-ABL1

42. b. The clinical presentation of disseminated intravascular

coagulation (DIC), along with the peripheral

smear findings, are consistent with acute promyelocytic

leukemia (AML with t[15:17]; PMLRARa).

Acute lymphoblastic leukemia (ALL) is

unlikely because of the suggestion of a disorder

of the myeloid line because of the presence of Auer

rods. AML with t(9:11); MLLT3-MLL is a disorder

involving the monocytic line, although DIC

may be associated with this disorder.

43. A patient presents with an elevated WBC count,

increased monocytes, and blasts present on the

differential. Flow cytometry is performed with the

following results: CD4+, CD11b+, CD11c+, CD13+,

CD14+, CD33+, CD36+, CD64+.Which of the following

diagnoses is the most likely?

a. AML with minimal differentiation

b. AML with maturation

c. Acute myelomonocytic leukemia

d. Acute monoblastic leukemia

43. c. Acute myelomonocytic leukemia is the most likely

because of the presence of both myeloid (CD13,

CD33) and monocytic cell lines (CD4, CD14,

CD11b, CD11c, CD64, CD36).

44. A 75-year-old male patient visits his physician for an

annual checkup. His CBC showed an elevated WBC

count with numerous small lymphocytes and smudge

cells, and a subsequent bone marrow biopsy and

aspirate showed hypercellularity with increased lymphoid

cells. What is a presumptive diagnosis based

on this information?

a. Acute lymphoblastic leukemia

b. Chronic lymphocytic leukemia/small cell lymphocytic

lymphoma

c. Hairy cell leukemia

d. Therapy-related acute myelogenous leukemia

b. This smear and bone marrow picture is typical of

chronic lymphocytic leukemia (CLL) with numerous

mature small lymphocytes. Acute lymphocytic

leukemia (CALL)wouldshowthepresenceofblasts;

hairy cell leukemia shows a hypocellular, fibrotic

bone marrow with hairy lymphocytes. Acute myelogenous

leukemia (AML) is not indicated, because

there is no evidence of myeloid cells and blasts.

45. Which of the following is not considered a disorder

of plasma cells?

a. Monoclonal gammopathy of undetermined

significance

b. Multiple myeloma

c. Se´zary syndrome

d. Waldenstro¨ m's macroglobulinemia

c. Se´zary syndrome is a disorder of T lymphocytes,

whereas the others are plasma cell disorders.

Which of the following sets of CD markers are associated

with T lymphocytes?

a. CD2, CD3, CD4

b. CD13, CD14, CD15

c. CD19, CD20, CD22

d. CD34, CD71, CD117

a. CD2, CD3, CD4, CD5, CD7, and CD8 are associated

with T lymphocytes. CD13, CD14, CD15

are associated with granulocytic/monocytic cells.

CD19, CD20, CD22 are associated with B cells.

CD34 and CD117 are immature cell markers,

and CD71 is an erythroid marker.

47. Bone marrow cellularity is most often estimated by

examining which of the following?

a. Aspirate

b. Buffy coat

c. Core biopsy

d. Crush preparations

47. c. Bone marrow core biopsies are the best indicator

of bone marrow architecture and cellularity,

because they provide a visual representation of

the hematologic cells, fat, and vascular structure.

Although a general idea may be obtained by

examining the aspirate, it is better used for looking

at the specific cell morphology. The buffy

coat or concentrated smears concentrate any cells

present, particularly in cases of hypocellular

samples.

48. A dry tap may be seen in bone marrow aspirations in

all of the following conditions except:

a. Aplastic anemia

b. Hairy cell leukemia

c. Multiple myeloma

d. Primary myelofibrosis

c. Fibrotic or hypercellular marrow is seen in all of

the following except multiple myeloma, in which

sheets of plasma cells may be present.

9. The largest hematopoietic cells present in the bone

marrow are:

a. Lymphoblasts

b. Megakaryocytes

c. Osteoblasts

d. Pronormoblasts

b. Megakaryocytes, with diameters up to 50 mm, are

the largest cells present in a normal bone marrow

sample. Lymphoblasts and pronormoblasts are less

than 20 mm in diameter. Osteoblasts, although

large cells, are not hematopoietic cells, and are

used in the formation and modeling of bone.

50. Hemoglobin A contains which of the following configurations

of globin chains?

a. a2b2

b. a2d2

c. a2g2

d. a2e2

a. Hemoglobin (Hgb)A is characterized by pairs of a

and b chains, a2b2. a2d2 is Hgb A2, a2g2 is Hgb F,

and a2e2 is Hgb Gower 2, an embryonic Hgb.

51. Which of the following locations is not a site of extramedullary

hematopoiesis?

a. Bone marrow

b. Liver

c. Spleen

d. Thymus

51. a. The bone marrow is the site of intramedullary

hematopoiesis. The liver and spleen are sites of

hematopoietic activity in the embryo, and hematopoietic

activity may be renewed in cases of

bone marrow compromise, such as primary

myelofibrosis. The thymus is a site for lymphoid

development.

52. Patients with renal failure often exhibit compromised

hematopoietic activity because of which of

the following?

a. Concurrent depression of thyroid

hormones

b. Decreased production of erythropoietin

c. Decreased production of GM-CSF

d. Bone marrow suppression caused by medications

b. Erythropoiesis is stimulated by erythropoietin,

which is produced in the kidney, and renal failure

can decrease the production of erythropoietin.

53. Which of the following best describes the function of

the Rapoport-Luebering pathway?

a. It produces ATP to help maintain RBC membrane

deformability

b. It results in the reduction of glutathione

c. It produces 2,3 diphosphoglycerate

(2,3 DPG)

d. It produces cytochrome b reductase

c. The Rapoport-Luebering shunt is involved in the

production of 2,3 diphosphoglycerate, which

helps regulate oxygen delivery to the tissues.

The overall Embden-Meyerhof pathway is used

in the production of adenosine triphosphate

(ATP); the hexose-monophosphate shunt functions

in reducing glutathione; and the methemoglobin

reductase pathway is involved in the

reduction of methemoglobin.

A 3-year-old male patient visits the pediatrician

for a well-child checkup and routine CBC. He has

a total WBC count of 5.0 109/L, RBC count of

3.8 1012/L, and platelet count of 225 109/L. The

differential showed 25% segmented neutrophils,

62% lymphocytes, 10% monocytes, and 3% eosinophils.

This patient is likely:

a. A normal child

b. Suffering from an acute bacterial infection

c. Immunosuppressed

d. A patient with leukemia

a. This is most likely a normal child, because children

usually have higher relative lymphocyte

counts than adults. The patient has normal total

white blood cell, red blood cell, and platelet

counts and normal differential results.

55. Which of the following cell types exhibit IgE receptors

on their surface membranes?

a. Basophils

b. Eosinophils

c. Band neutrophils

d. Monocytes

a. Basophils have IgE receptors on their surface

membranes. Once IgE is bound to the receptor,

it allows the release of the cell's granule contents.Eosinophils, neutrophils, and monocytes all

contain granules; however, they are not associated

with IgE.

56. A 62-year-old female patient's CBC showed the following

results: total WBC count of 14.0 109/L,

RBC count of 3.95 1012/L, and platelet count of

245 109/L. The differential showed 65% segmented

neutrophils, 10% bands, 15% lymphocytes,

and 10% monocytes. Toxic granulation and Do¨ hle

bodies were seen in many of the neutrophils. Which

of the following is most likely?

a. The patient had just finished running a half

marathon

b. The patient has a bacterial infection

c. The patient is normal

d. The patient has a helminth infection

56. b. The patient most likely has a bacterial infection,

because the white blood cell (WBC) count is

slightly elevated with increased neutrophils,

including the presence of 10% bands. Do¨ hle bodies

and toxic granulation, although not exclusive

to bacterial infections, are toxic neutrophil

changes that may present in these cases. Strenuous

exercise may lead to a transient elevation in

WBCs and the mobilization of neutrophils from

the marginating to circulating pools; however,

toxic granulation and Do¨ hle bodies are not usually

seen. If the patient had a parasitic infection,

elevated numbers of neutrophils would be

expected.

57. A CBC on a patient with Chediak-Higashi syndrome

is expected to exhibit which of the following?

a. Giant platelets and Do¨ hle-like inclusions in the

cytoplasm of all granulocytes

b. Large, darkly staining cytoplasmic granules in

all WBCs

c. Giant fused granules and lysosomes in WBC

cytoplasm

d. Leukocytosis and bilobed eosinophils

57. c. Chediak-Higashi syndrome is characterized by

giant fused granules in the white blood cell cytoplasm,

with neutropenia and thrombocytopenia

as the disease progresses, and patients often die

in infancy or early childhood because the granules

normally released to aid in the killing of bacteria

cannot be released to aid in the kill process. A

complete blood count with giant platelets and

Do¨ hle-like inclusions in the granulocytes is more

characteristic of the May-Hegglin anomaly, and

large, dark granules are more associated with

Alder-Reilly anomaly.

58. Patients with infectious mononucleosis often have

the following CBC results:

a. Lymphocytosis, including increased variant/

reactive lymphocytes

b. Lymphocytopenia with numerous small

lymphocytes

c. Neutrophilia, including a predominant shift to

the left

d. Neutropenia with a distinct predominance of

toxic granulation

a. Patients with infectious mononucleosis often

exhibit an increase in lymphocytes, along with

the presence of reactive lymphocytes. Neutrophilia

with a left shift is typically seen in bacterial

infections or other acute infections.

59. Flow cytometry for monitoring a patient with acquired

immunodeficiency syndrome should include

markers for which of the following?

a. CD30 and CD42

b. CD4 and CD8

c. CD34 and CD33

d. CD21 and CD22

b. CD4 and CD8 markers are monitored in patients

with acquired immunodeficiency virus infection.

CD33 and CD34 would more likely be used for

investigating a suspected acute myeloid leukemia

(AML) case.

60. Which of the following disorders is classified as a

myelodysplastic/myeloproliferative disease?

a. Acute promyelocytic leukemia

b. Chronic lymphocytic leukemia

c. Atypical chronic myelogenous leukemia

d. Essential thrombocythemia

c. Atypical chronic myelogenous leukemia is classified

as an MDS/MPD, because it has characteristics

of both disorders. Acute promyelocytic

leukemia is an acute leukemia, and chronic lymphocytic

leukemia is a chronic lymphoid disorder

that affects a different cell line than seen in MDS/

MPD. Essential thrombocythemia is classified as a

myeloproliferative disorder.

All of the following cells are derived from

CFU-GEMM, common myeloid progenitor cells

except:

a. Basophils

b. Lymphocytes

c. Neutrophils

d. RBCs

b. Lymphocytes are derived from the common lymphocyte

progenitor cell, whereas the other cells

are derived from the common myeloid progenitor

(or CFU-GEMM).

62. A patient's differential count shows an elevated

eosinophil count. This is consistent with which of

the following?

a. Aplastic anemia

b. Bacterial infection

c. Parasitic infection

d. Viral infection

62. c. Elevated eosinophil counts are often seen in parasitic

infections, particularly those caused by helminths.

Aplastic anemia shows decreases in all

cell counts. Bacterial infections tend to have

increased neutrophil numbers, and viral infections

tend to have increased lymphocytes.

Antibodies are produced by which of the following:

a. Macrophages

b. T lymphocytes

c. Plasma cells

d. Basophils

63. c. Antibodies are produced by lymphocytes, specifically

B cells in the form of plasma cells. None of

the other cells can produce antibodies.

64. The nitroblue tetrazolium reduction test is used to

assist in the diagnosis of:

a. Leukocyte adhesion disorders (LADs)

b. Chronic granulomatous disease (CGD)

c. May-Hegglin anomaly

d. Pelger-Huet anomaly

64. b. The nitroblue tetrazolium test will reduce nitroblue

tetrazolium in normal neutrophils. In cases

of chronic granulomatous disease (CGD), the

phagocytic cells cannot make nicotinamide adenine

dinucleotide phosphate (NADPH) oxidase,

which is used in the kill mechanism of neutrophils.

Leukocyte adhesion deficiency (LAD) involves

problems with adhesion to endothelial cells, and

both May-Hegglin and Pelger-Huet anomalies

have normal neutrophil phagocytic function.

65. A newly diagnosed patient has an acute leukemia.

Which of the following would initially be the

mostuseful indetermining the origin of the blasts seen?

a. Leukocyte alkaline peroxidase (LAP) and nonspecific

esterase (NSE)

b. Periodic acid-Schiff (PAS) and tartrate-resistant

acid phosphatase (TRAP)

c. Myeloperoxidase (MPO) and terminal dexoynucleotidyl

transferase (TdT)

d. Sudan black B and brilliant cresyl blue

c. Myeloperoxidase (MPO) and terminal deoxnucleotidyl

transferase (TdT) would be good initial

markers to use. MPO is positive in myeloblasts

and promyelocytes, and TdT is positive in early

lymphoid cells. Leukocyte alkaline peroxidase

(LAP) is used for mature neutrophil activity, nonspecific

esterase (NSE) is positive in monocytic

cells, Sudan black B is used only for myeloid cells,

and brilliant cresyl blue is not used to determine

blast origin.

66. Therapy for CML often includes the use of a targeted

tyrosine kinase inhibitor, such as:

a. Imatinib mesylate

b. All-trans retinoic acid

c. Ablative chemotherapy

d. 2-CDA/cladribine

66. a. Imatinib mesylate and related drugs are used to target

p210 formed as a result of the BCR/ABL fusion

gene in chronic myelogenous leukemia (CML). Alltrans

retinoic acid therapy is often used in cases of

acute myeloid leukemia (AML), and 2-CDA/

cladribine is a drug used for hairy cell leukemia.

A 58-year-old female was seen by her physician for

increasing fatigue. Her CBC shows the following

results:

WBC 15.5 109/L

RBC 5.90 1012/L

Hgb 17.5 g/dL

Hct 53.0%

Platelet 425 109/L

Differential:

55% segmented neutrophils,

3% bands,

30% lymphocytes,

9% monocytes,

1% eosinophils,

2% metamyelocytes;

RBC

and platelet

morphology appear

normal

Which of the following conditions is the most likely

cause of these results?

a. Chronic myelogenous leukemia

b. Polycythemia vera

c. Acute bacterial infection

d. The patient is normal

67. b. This is most likely a case of polycythemia vera,

because the patient exhibits more than two of

the diagnostic criteria required by the World

Health Organization (hemoglobin >16.5 g/dL,

platelet count >400 109/L, and, additionally,

white blood cell count >12 109/L, although

it's not apparent from the information given

whether an infection is present). Chronic myelogenous

leukemia is less likely because of the lower

numbers of immature neutrophils and the higher

red blood cell count. Although bacterial infection

is not ruled out from this information, the combination

of the other elevated parameters is usually

seen in cases of polycythemia vera.

68. Polycythemia vera can be differentiated from

secondary polycythemia because of polycythemia

vera presenting with which of the following?

a. Elevated hemoglobin results

b. Decreased erythropoietin levels

c. Normal to decreased WBC counts

d. Erythroid hyperplasia in the marrow

b. Polycythemia vera differs from secondary polycythemia

because it exhibits elevated red blood cell counts while the erythropoietin levels are

decreased. Secondary polycythemia shows elevated

erythropoietin levels, often as a response

to tissue hypoxia resulting from the patient's initial

condition.

69. The genetic mutation associated with CML is:

a. t (15;17)(q22;q12)

b. t(11;14)(p15;q11)

c. t(9:22)(q34;q11.2)

d. t(8:21)(q22;q22)

69. c. The mutation seen in chronic myelogenous leukemia

(CML) is t(9;22)(q34;11.2), resulting in the

Philadelphia chromosome, with the translocation

creating a fusion gene, BCR-ABL. t (15;17)(q22;

q12) is seen in acute promyelocytic leukemia, t

(8:21)(q22;q22) is seen in acute myeloid leukemia

(AML) with maturation, and t(11;14)(p15;q11) is

seen in precursor T-cell acute lymphoblastic

leukemia.

70. Which of the following is not classified as a myeloproliferative

neoplasm?

a. Chronic eosinophilic leukemia

b. Essential thrombocythemia

c. Mastocytosis

d. Waldenstro¨ m's macroglobulinemia

d. All are myeloproliferative neoplasms with the

exception of Waldenstro¨m's macroglobulinemia,

which is a plasma cell disorder.

71. What is the minimum percentage of ringed sideroblasts

present in the bone marrow for a diagnosis

of refractory anemia with ringed sideroblasts?

a. 10%

b. 15%

c. 20%

d. >25%

71. b. More than 15% ringed sideroblasts must be present

in the bone marrow for a diagnosis of refractory

anemia with ringed sideroblasts (RARS).

Ringed sideroblasts must have at least five iron

granules, surrounding at least one third of the

nucleus of a nucleated red blood cell.

72. All of the following are considered to be signs of dyserythropoiesis

except:

a. Multinucleate RBCs

b. Basophilic stippling

c. Do¨ hle bodies

d. Oval macrocytes

72. c. Do¨ hle bodies are found in neutrophils.

Multinucleate red blood cells (RBCs), nuclear

bridging, basophilic stippling, siderotic granules,

and macroovalocytes may be seen in dyserythropoietic

RBCs.

73. Features of dysmyelopoiesis and dysmegakaryopoiesis

seen on a peripheral smear or bone marrow in

cases of myelodysplastic syndromes include all of

the following except:

a. Pelgeroid neutrophils

b. Neutrophils showing hypogranulation

c. Giant abnormal platelets with abnormal granules

d. Siderotic granules

73. d. Although iron granules may be seen in some myelodysplastic

syndromes, siderotic granules are

found in red blood cells, not in neutrophils or

platelets. Pelgeroid nuclei in neutrophils and

abnormal granulation patterns in neutrophils and

platelets are features of dyspoiesis of myeloid cells

and platelets.

74. The peripheral blood and bone marrow picture

sometimes will look similar in myelodysplastic syndromes

and some RBC disorders. Which of the following

RBC disorders tends to have a peripheral

smear appearance similar to cases of myelodysplastic

syndromes?

a. Iron deficiency anemia

b. a-Thalassemia minor

c. Megaloblastic anemia

d. Warm autoimmune hemolytic anemia

74. c. Megaloblastic anemia, resulting from a deficiency

of vitamin B12 or folic acid can look similar to

myelodysplastic syndrome, because decreased

vitamin B12 or folic acid can lead to a similar

appearance, such as nuclear-to-cytoplasmic asynchrony

and a hypercellular dysplastic bone marrow.

Iron-deficiency anemia and a-thalassemia

minor exhibit microcytic/hypochromic cells with

relatively normal white blood cells (WBCs), and

warm autoimmune hemolytic anemia is a hemolytic

anemia with normal WBCs, although red

blood cells usually show macrocytosis, polychromasia,

and spherocytes.

Most of the chromosome abnormalities seen in myelodysplastic

syndrome involve which of the following

chromosomes?

a. 5, 7, 8, 11, 13, 20

b. 2, 3, 9, 15, 16, 26

c. 3, 6, 10, 14, 21

d. 1, 4, 15, 17, 21

a. The most common abnormalities of chromosomes

in myelodysplastic syndrome (MDS) occur in chromosomes 5, 7, 8, 11, 13, and 20. Although

multiple chromosome abnormalities have been

implicated in MDS, currently 5q syndrome is

the only abnormality specific to one disorder subtype

in the World Health Organization 2008

classification.

76. Whichof the followingisnotoneof therecurrentgenetic

abnormalities seen in cases of acute myeloid leukemia?

a. AML with t(8;21)(q22;q22); AML1(CBFa)/ETO

b. AML with t(15;17)(q22;q12); (PML/RARa)

c. AML with inv(16)/p(13;q22); (CBFb/MYH11)

d. AML with t(1;19)(q23;q13); (E2A/PBX1)

d. All are recurrent genetic abnormalities in acute

myeloid leukemia (AML) except AML with t

(1;19)(q23;q13), (E2A/PBX1), which is an abnormality

seen in some cases of precursor B-cell ALL.

77. AML with 11q23 (MLL) abnormalities are associated

with which cell line?

a. Eosinophil

b. Erythrocyte

c. Monocyte

d. Neutrophil

c. Monocytic precursors are associated with 11q23

(MLL) abnormalities. Eosinophils are associated

with inv(16)(p13;q22) and t(16;16)(p13;q22),

whereas neutrophils are associated with a variety

of other genetic abnormalities. Erythrocytes are

currently not associated with a specific generality.

78. T-cell ALL most commonly affects which of the

following?

a. Infants

b. Teenaged males

c. Adult females

d. Elderly males

b. T-cell acute lymphoblastic leukemia (ALL) most

commonly affects teenaged males who present

with a mediastinal mass, although it also may

occur in adult patients in some cases. B-cell

ALL is typically seen in children.

79. Which of the following disorders is considered to be

classified by WHO as an AML, not otherwise

classified?

a. Acute erythroid leukemia

b. Acute megakaryoblastic leukemia

c. Acute promyelocytic leukemia

d. AML without maturation

c. Acute promyelocytic leukemia falls under the classification

of acute myeloid leukemia (AML) with

recurrent cytogenetic abnormalities, because it

manifests with t(15;17)(q22;q12), (PML/RARa).

The others currently do not have a specific recurrent

cytogenetic abnormality and all are included

in the "not otherwise classified" category.

80. A 69-year-old female patient presented with symptoms

of fatigue and easy bruising.ACBCwas ordered.

The peripheral smear showed a large number of

blasts, anemia, and thrombocytopenia. A bone marrow

examination was performed, revealing hypercellularity

and a blast appearance similar to that of the

peripheral smear. Flow cytometry revealed cells positive

for CD 13, CD 33, CD 34, CD 38, CD 117, and

HLA-DR. Cells were negative for TdT, myeloperoxidase,

and nonspecific esterase. Based on this information,

which of the following is most likely?

a. AML with minimal differentiation

b. AML without maturation

c. B-cell ALL without maturation

d. Acute monoblastic leukemia

a. This is a case of acute myeloid leukemia (AML)

with minimal differentiation. Early myeloid cell

precursors and stem cell markers are present,

while the patient is negative for terminal deoxynucleotidyl

transferase (TdT), which rules out

lymphoid involvement. The patient also is negative

for myeloperoxidase and nonspecific esterase,

in the presence of the early myeloid CD

markers, which is consistent with AML with

minimal differentiation. AML without maturation

would have a similar flow cytometry profile,

but would have a positive myeloperoxidase

result.

A 3-year-old female patient was having symptoms of

lethargy and bruising and reported pain in her legs.

Her mother also mentioned noticing several swollen

lymph nodes when bathing the child. The pediatrician

ordered a CBC, which had the following results.

WBC 18.5 1012/L

RBC 3.00 1012/L

Hgb 9.0 g/dL

Hct 27.0%

MCV 90 fL

MCH 30 pg

MCHC 33%

Platelet 58 109/L

Differential:

80% blastocytes,

6% segmented neutrophils,

8% lymphocytes,

6% monocytes.

RBC morphology was normal, and

platelets were markedly

decreased. What is the most likely reason that the physician

ordered a lumbar puncture after receiving the

CBC results?

a. To rule out an acute case of meningitis

b. To look for leukemia cells in the spinal fluid

c. To rule out infectious mononucleosis

d. To rule out multiple sclerosis

b. The lumbar puncture would be ordered to determine

if there was leukemic involvement in the

central nervous system (CNS), because the

patient's other results are consistent with possible

acute myeloid leukemia (ALL). If blasts are seen

in the CNS, intrathecal chemotherapy would be

indicated in addition to a standard therapy regimen.

Orders for a bone marrow examination

and flow cytometry are also indicated. Blasts

should not be seen in the peripheral blood in meningitis, infectious mononucleosis, or multiple

sclerosis. If the physician suspected infectious

mononucleosis, serologic examination specific

to infectious mononucleosis would be ordered,

not a lumbar puncture.

A 78-year-old man was previously diagnosed with

chronic lymphocytic leukemia (CLL). Periodic

CBCs were ordered, and several months of CBCs

maintained an appearance consistent with cases

of CLL.

WBC 58.5x1012/L

RBC 3.90x 1012/L

Hgb 12.0 g/dL

Hct 36.0%

MCV 92 fL

MCH 3 pg

MCHC 33%

Platelet 132x109/L

Differential: 70%

lymphocytes, 8%

segmented

neutrophils, 2%

monocytes, 20%

unidentified cells with

lymphoid appearance

and a prominent

nucleolus

Which of the following is most likely?

a. The patient has developed Se´zary syndrome

b. Thepatienthasdevelopedprolymphocytic leukemia

c. The patient has developed multiple myeloma

d. The patient now has a concurrent case of CLL

and ALL

b. The patient most likely has Prolymphocytic leukemia

(PLL), because prolymphocytes typically

look like lymphocytes with a prominent nucleolus.

Se´zary syndrome has lymphoid cells with a

convoluted nucleus, and plasma cells are the cells

seen in multiple myeloma.

Multiple myeloma exhibits laboratory features

except which of the following?

a. Occasional plasma cells in the peripheral blood

b. Rouleaux

c. Hypercalcemia

d. Decreased immunoglobulin

83. d. Multiple myeloma shows all of the following

laboratory features except for decreased immunoglobulin.

Immunoglobulin is increased, because

the disorder is a monoclonal gammopathy, with

increased IgG.

84. The diagnostic cell type seen in Hodgkin lymphoma

is:

a. Binucleate plasma cell

b. Reed Sternberg cell

c. Bence Jones lymphocyte

d. Burkitt lymphocyte

84. b. Reed Sternberg cells, sometimes described as

"owl eye" cells, are present in cases of classic

Hodgkin lymphoma. Plasma cells are seen in

multiple myeloma and other plasma cell

neoplasms.

85. Which of the following appearances describes the

types of cells seen in Se´zary syndrome?

a. Plasma cells containing immunoglobulin deposits

b. Large circulating micromegakaryocytes

c. Lymphocytes with convoluted, cerebriform

nuclei

d. Prolymphocytes with prominent azurophilic

granules

85. c. Se´zary cells are lymphoma cells characterized by

cerebriform nuclei and irregular nuclear outlines.

Which of the following best describes the function of

the hexose-monophosphate pathway?

a. It produces ATP to help maintain RBC membrane

deformability

b. It results in the reduction of glutathione

c. It produces 2,3 diphosphoglycerate (2,3 DPG)

d. It produces cytochrome b reductase

86. b. The hexose monophosphate pathway is used to

reduce glutathione, which is used to help the cell

combat oxidative damage. Adenosine triphosphate

(ATP) is produced via the Embden-

Meyerhof pathway. 2,3 diphosphoglycerate (2,3

DPG) results from the Rapoport-Luebering

pathway.

87. A patient has a reticulocyte count of 3.5%. This

shows which of the following?

a. Bone marrow response in producing more RBCs

because of increased need

b. A normal reticulocyte count

c. Patient transfusion of whole blood

d. Lack of response to vitamin therapy after a diagnosis

of iron-deficiency anemia

a. A normal reticulocyte count is 0.5% to 1.5%.

Values above 1.5% show the patient is responding

to an increased need for red blood cells (RBCs) by

pushing out increased numbers of immature

RBCs to the bone marrow.

88. Which of the following cases does not warrant a bone

marrow examination?

a. Presence of blasts on the peripheral smear

b. Postchemotherapy assessment for minimal residual

disease

c. Diagnosis of iron-deficiency anemia

d. Diagnosis of suspected systemic fungal infection

88. d. Bone marrow examination is not indicated in the

diagnosis of a standard case of iron deficiency, in

which diagnosis can be made by using less invasive

measures such as peripheral blood indices

and serum iron chemistries.

89. A bone marrowsample for a patient with newly diagnosed

chronic myelogenous leukemia would often be

expected to have an M/E ratio of:

a. 1:1

b. 2:1

c. 1:2

d. 10:1

d. The myeloid-to-erythroid (M/E) ratio looks at

the number of myeloid cells (M) to nucleated

erythroid cells (E) in the bone marrow. A ratio

of 1:1 would mean the patient was producing

approximately the same number of red blood

cells (RBCs) and myeloid cells. A ratio of 2:1

is in the normal range. A ratio of 1:2 indicates

the patient is showing erythroid hyperplasia,

producing larger numbers of nucleated RBCs than white blood cells, which is often seen in

ineffective erythropoiesis. Patients with chronic

myelogenous leukemia (CML) will have elevated

M/E ratios, because they are producing

large numbers of myeloid cells with the malignant

cell clone.

90. Which of the following is not implicated as a cause of

nonmegaloblastic macrocytic anemia?

a. Alcoholism

b. Hemochromatosis

c. Hypothyroidism

d. Liver disease

b. Macrocytosis without megaloblastosis can present

in patients with liver disease, chronic alcoholism,

and hypothyroidism and in other cases

in which reticulocyte production is elevated.

Hemochromatosis is a disorder that leads to elevated

red blood cell production, not anemia.

91. Which of the following results is consistent with a

diagnosis of aplastic anemia?

a. Hypocellular bone marrow, absolute neutrophil

count of 0.5 109/L, platelet count of 40 109/L,

Hgb 8 g/dL

b. Hypocellular bone marrow, absolute neutrophil

count of 2.5 109/L, platelet count of 75 109/L,

Hgb 10 g/dL

c. Hypercellular bone marrow, absolute neutrophil

count of 1.5 109/L, platelet count of

100 109/L, Hgb 14 g/dL

d. Hypocellular bone marrow, absolute neutrophil

count of 0.5 109/L, platelet count of 90 109/L,

Hgb 11 g/dL

a. Diagnostic criteria for aplastic anemia include a

hypocellular bone marrow, absolute neutrophil

count less than 1.5 109/L, platelet count less

than 50 109/L, and hemoglobin value less than

10 g/dL, with decreased reticulocyte response

(Rodak, 2012).

92. The following statement is true of mutations in athalassemia

compared to those seen in bthalassemia:

a. Mutations in a-thalassemia occur as a result

of reduced or absent expression of the globin gene

b. Mutations in a-thalassemia occur as a result of

the deletion of one or more globin genes

c. The a-globin gene is expressed on chromosome 11

d. The b-globin gene is expressed on chromosome 16

b. Mutations in a-thalassemia occur as a result of

deletions of one or more a-globin genes, and

mutations in b-thalassemia occur because of

reduced or absent expression of one or more bglobin

genes. The a-globin gene is found on chromosome

16, and the b-globin gene is found on

chromosome 11.

A patient's genotype is a/ a. This patient will have

a CBC that shows which of the following?

a. Decreased RBC count with numerous

target cells

b. Decreased RBC count with microcytic/

hypochromic RBCs

c. Increased RBC count with normal RBCs

d. Increased RBC count with microcytic/

hypochromic RBCs

d. This is a patient with a-thalassemia minor. An

increased red blood cell (RBC) count would be

expected with hemoglobin, mean corpuscular

volume, and mean corpuscular hemoglobin

values lower than expected for the number of

red blood cells present.

94. Patients with sickle cell anemia and b-thalassemia

major may not show clinical symptoms until the

patient is at least 6 months of age because of which

of the following?

a. The mutations are acquired after the child is born

b. The mutations are activated by dietary and

maternal factors

c. Themutationsmay notmanifest clinically at birth

because the presence of hemoglobin F decreases

d. The mutations lead to elevations in a genes that

compensate for the decreased gene expression

c. Hemoglobin (Hgb) F is present in larger quantities

in fetal development and after a baby is born. As

the baby makes more b-globin chains than g-globin

chains, usually between 6 and 9 months of age, the

Hgb F decreases and the expression of the missing

or mutated gene becomes clinically apparent.

95. The thymus is a site used as a maturation compartment

for:

a. B cells

b. T cells

c. Megakaryocytes

d. Monocytes

b. The thymus is associated with lymphocyte development

for precursor T cells, whereas the others

all develop in the bone marrow.

96. A manual hemocytometer count was required to

check a patient's total WBC count. A 1:20 dilution

was made and used when the four large "W"

squares were counted on both sides of the hemacytometer.

A total of 105 cells were counted between

the two sides. What was the patient's total WBC

count?

a. 0.33 109/L

b. 2.1 109/L

c. 2.6 109/L

d. 5.3 109/L

96. c. The equation used is number of cells counted/

(number of squares counted area of square

depth of square) reciprocal dilution to get

cells/mL. Cells/mL 106/mL /L will convert the

answer to cells per liter. For this patient, 105/(8

1 mm2 0.1 mm) 20/1.2625 cells/mL,

which converts to 2.6 109/L.

97. Hereditary elliptocytosis results from defects in

which of the following?

a. Ankyrin

b. Band 3 protein

c. Spectrin

d. Pyruvate

c. Hereditary elliptocytosis results from an autosomal

dominant mutation in the spectrin proteins and protein 4.1R, which will lead to

instability in the red blood cell membrane. Spectrin

mutations may also appear in hereditary

spherocytosis, in addition to mutations of several

other proteins.

98. Primary neutrophil granules contain:

a. Acetyltransferase, collagenase, gelatinase, lysozyme,

b2-microglobulin

b. Alkaline phosphatase, cytochrome b558, complement

receptor 1, complement 1q receptor,

vesicle-associated membrane-2

c. b2-Microglobulin, collagenase, gelatinase lactoferrin,

neutrophil gelatinase-associated lipocalin

d. Acid b-glycerophosphatase, cathespins, defensins,

elastase, myeloperoxidase, proteinase-3

98. d. Acid b-glycerophosphatase, cathespins, defensins,

elastase, myeloperoxidase, and proteinase-

3 are found in the primary granules of neutrophils.

b2-Microglobulin, collagenase, gelatinase lactoferrin,

and neutrophil gelatinase-associated

lipocalin are found in the secondary (specific

granules). Acetyltransferase, collagenase, gelatinase,

lysozyme, b2-microglobulin are found in tertiary

granules. Alkaline phosphatase, cytochrome

b558, complement receptor 1, complement 1q

receptor, vesicle-associated membrane-2 are

found in the secretory granules.

A 36-year-old man visited the emergency department

because of alternating episodes of fever and

chills that persisted over several days. The patient

stated he had not felt well since returning from a

mission trip to Africa. The physician ordered a

CBC with the following results.

WBC 3.5 109/L

RBC 3.80 1012/L

Hgb 12.0 g/dL

Hct 36.0%

MCV 95 fL

MCH 32 pg

MCHC 33%

Platelet

145 109/L

Differential: Normal WBC

distribution, normocytic

normochromic RBCs

with some inclusions

present and several

abnormal platelet-like

structures shaped like

boomerangs

What should be done with this sample next?

a. Rerun the sample to make sure it is not

clotted

b. Clean the stainer and make another slide to

examine

c. Refer the sample to the pathologist for further

identification

d. Report the results, because the results are

normal

99. c. This sample, based on the intracellular red blood

cell inclusions and boomerang-shaped or bananashaped

structures is suspicious for a malarial

infection, particularly based on the patient travel

history. The sample should be referred to the

pathologist or technical supervisor for confirmation

and speciation.

100. Patients with suspected paroxysmal cold hemoglobinuria

can be confirmed by performing which of

the following?

a. Direct antiglobulin test (DAT)

b. Donath-Landsteiner test

c. Osmotic fragility test

d. G6PD activity assay

100. b. The Donath-Landsteiner test can be used to

demonstrate proximal cold hemoglobinuria by

identifying glucose-6-phosphate dehydrogenase

deficiency. The osmotic fragility test and direct

antiglobulin test (DAT) would be less useful

for differential diagnosis. G6PD activity is used

to identify cases of glucose-6-dehydrogenase

(G6PD) deficiency.