HEMATOLOGY

Question 1

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

Answer 1

c. Serum ferritin

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.

Question 2

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 x 10^12/L
Hgb: 6.2 g/dL
Hct: 18.8%
MCV: 75.2 fL
MCH: 24.8 pg
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

Answer 2

a. Iron-deficiency anemia

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.

Question 3

A peripheral smear shows a decreased RBC count
with microcytic, hypochromic cells with small grape-like inclusions in the RBCs on both Wright stain and Prussian blue stain. This is consistent with:
a. Iron-deficiency anemia
b. Sideroblastic anemia
c. Pernicious anemia
d. b-Thalassemia minor

Answer 3

b. Sideroblastic anemia

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. Iron-deficiency 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.

Question 4

Given the following results of iron studies, which disorder is the most likely?

↓ Serum iron
↓ Ferritin
↑ TIBC
↓ Saturation

a. Iron-deficiency anemia
b. Sideroblastic anemia
c. Anemia of chronic inflammation
d. Hemochromatosis

Answer 4

a. Iron-deficiency anemia

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.

Question 5

Acquired sideroblastic anemia may be present in all of the following except:
a. Alcoholism
b. Lead poisoning
c. Malabsorption
d. Myelodysplastic syndromes

Answer 5

c. Malabsorption

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).

Question 6

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

Answer 6

b. Intrinsic factor antibodies

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.

Question 7

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

Answer 7

b. Deficiencies in Vitamin B12 and folic acid

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.

Question 8

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

Answer 8

c. Pernicious anemia

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.

Question 9

The CBC for a 57-year-old man had the following results. Which tests would be best to order next?

RBC: 2.50 x 10^12/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

Answer 9

b. Vitamin B12 and folic acid levels

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.

Question 10

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

Answer 10

a. Unknown causes

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.

Question 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

Answer 11

c. Absolute lymphocyte count

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.

Question 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

Answer 12

a. Increased chromosome fragility

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.

Question 13

Which of the following is decreased in cases of intravascular hemolytic anemia?
a. Bilirubin
b. Urine hemosiderin
c. Haptoglobin
d. Plasma hemoglobin

Answer 13

c. Haptoglobin

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.

Question 14

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

Answer 14

b. Macrocytic, normochromic cells with increased polychromasia

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.

Question 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

Answer 15

d. Anemia of chronic disease

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

Question 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

Answer 16

b. DAT

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.

Question 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

Answer 17

a. Negative for CD55 and CD59

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.

Question 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

Answer 18

a. Exposure to oxidant damage

Glucose-6-dehydrogenase (G6PD) deficiency shows lack of enzyme activity that is needed for the reduction of glutathione, which in turn works 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.

Question 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

Answer 19

c. Traumatic cardiac hemolytic anemia

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 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.

Question 20

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 x 10^9/L
RBC: 3.00 x 10^12/L
Hgb: 8.0 g/dL
Hct: 25.0%
MCV: 83 fL
MCH: 26 pg
MCHC: 32%
Platelets: 18 x 10^9/L
Differential: Normal WBCs
with moderate schistocytes
and polychromasia
PT: 12.8 secs
aPTT: 34 secs

a. Sickle cell anemia
b. Chronic myelogenous leukemia
c. Disseminated intravascular coagulation
d. Thrombotic thrombocytopenic purpura

Answer 20

d. Thrombotic thrombocytopenic purpura

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).

Question 21

Warm autoimmune hemolytic anemia is usually
caused by which of the following?
a. IgA antibodies
b. IgG antibodies
c. IgM antibodies
d. Complement

Answer 21

b. IgG antibodies

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

Question 22

Which of the following conditions is not associated
with secondary warm autoimmune hemolytic anemia?
a. CLL
b. Idiopathic onset
c. Rheumatoid arthritis
d. Viral infections

Answer 22

b. Idiopathic onset

Idiopathic onset is an unknown cause of warm autoimmune hemolytic anemia (WAIHA). Secondary WAIHA is usually associated with chronic lymphoid disorders, viral infections, and autoimmune disorders.

Question 23

The mutation seen in sickle cell anemia is:
a. β6 Glu → Val
b. β6 Glu → Lys
c. β26 Glu → Lys
d. β63 Glu → Arg

Answer 23

a. β6 Glu → Val

β6 Glu → Val is the mutation seen in sickle cell anemia. β6 Glu → Lys is the mutation seen in hemoglobin (Hgb) C, β26 Glu → Lys is the mutation seen in Hgb E, and β63 Glu → Arg is seen in Hgb Zurich.

Question 24

The majority of hospitalizations associated with sickle cell anemia are due to:
a. Cardiomegaly
b. Cholelithiasis
c. Pneumonia
d. Vasoocclusion

Answer 24

d. Vasoocclusion

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.

Question 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

Answer 25

a. Normocytic, normochromic RBCs with occasional target cells 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.

Question 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

Answer 26

b. Hemoglobin electrophoresis Hemoglobin (Hgb) electrophoresis, high-performance 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 show the presence of sickle cells; however, it will not determine if Hgb S is present in the case of someone heterozygous for Hgb S.

Question 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

Answer 27

b. Hemoglobin C 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.

Question 28

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 x 10^9/L RBC: 6.00 x 10^12/L Hgb: 10.0 g/dL Hct: 30.0% MCV: 60 fL MCH: 20 pg MCHC: 33% Platelet: 218 x 10^9/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 β-thalassemia minor d. There is a specimen quality issue because of a cold agglutinin

Answer 28

c. This patient is likely to have β-thalassemia minor 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.

Question 29

Hemoglobin H disease is described as: a. --/-α b. -α/-α c. --/ββ d. -β/-β

Answer 29

a. --/-α Hemoglobin H is the result of a three-gene deletion on the a gene. A two-gene deletion would result in a thalassemia minor.

Question 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. α-Thalassemia major b. β-Thalassemia major c. α-Thalassemia minor d. Hemoglobin H disease

Answer 30

b. β-Thalassemia major This is likely a case of β-thalassemia because hemoglobin (Hgb F) (α2γ2) and Hgb A2 (α2δ2) are increased. Thus a chains are able to be produced; however, β chains are lacking so no Hgb A (α2β2) is present. The other disorders listed are all covered in the α-thalassemia group.

Question 31

A 36-year-old male patient has a CBC performed as part of a routine work physical. The WBC count was 6.5 x 10^9/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

Answer 31

c. Pelger-Huet anomaly 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

Question 32

A 38-year-old male patient has the following CBC results: WBC: 32.5 x 10^9/L RBC: 5.50 x 10^12/L Hgb: 16.0 g/dL Hct: 48.0% Platelet: 225 x 10^9/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

Answer 32

a. Bacterial infection 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.

Question 33

Which of the following cytochemical stains is best used to distinguish cells of monocytic origin? a. α-Naphthyl acetate esterase b. Naphthol AS-D chloroacetate esterase c. Myeloperoxidase d. Periodic acid–Schiff

Answer 33

a. α-Naphthyl acetate esterase α-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.

Question 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

Answer 34

c. Hairy cell leukemia 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.

Question 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

Answer 35

b. JAK2 V617F 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.

Question 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

Answer 36

b. Karyotyping for the Philadelphia chromosome 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.

Question 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

Answer 37

a. Bone marrow biopsy 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.

Question 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

Answer 38

d. Decreased vitamin B12 and folic acid 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.

Question 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

Answer 39

c. Chronic Myelomonocytic Leukemia All of these disorders are classified as myelodysplastic syndromes with the exception of chronic myelomonocytic leukemia (CML), which was moved into the World Health Organization classification of myelodysplastic syndrome/myeloproliferative neoplasms, along with juvenile myelomonocytic leukemia, and atypical CML.

Question 40

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

Answer 40

a. Intermediate B-cell ALL 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.

Question 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 x 10^9/L c. The Philadelphia chromosome is present d. The patient is hypodiploid

Answer 41

a. The patient is a child 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.

Question 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-RARα c. ALL with t(12;21)(p13;q22); ETV6-RUNX1 d. ALL with t(9;22)(q34;q11.2); BCR-ABL1

Answer 42

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

Question 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

Answer 43

c. Acute myelomonocytic leukemia 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).

Question 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

Answer 44

b. Chronic lymphocytic leukemia/small cell lymphocytic lymphoma This smear and bone marrow picture is typical of chronic lymphocytic leukemia (CLL) with numerous mature small lymphocytes. Acute lymphocytic leukemia (CALL) would show the presence of blasts; 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.

Question 45

Which of the following is not considered a disorder of plasma cells? a. Monoclonal gammopathy of undetermined significance b. Multiple myeloma c. Sezary syndrome d. Waldenstrom’s macroglobulinemia

Answer 45

c. Sezary syndrome Sezary syndrome is a disorder of T lymphocytes, whereas the others are plasma cell disorders.

Question 46

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

Answer 46

a. CD2, CD3, CD4 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.

Question 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

Answer 47

c. Core biopsy 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.

Question 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

Answer 48

c. Multiple myeloma Fibrotic or hypercellular marrow is seen in all of the following except multiple myeloma, in which sheets of plasma cells may be present.

Question 49

The largest hematopoietic cells present in the bone marrow are: a. Lymphoblasts b. Megakaryocytes c. Osteoblasts d. Pronormoblasts

Answer 49

b. Megakaryocytes 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.

Question 50

Hemoglobin A contains which of the following configurations of globin chains? a. α2β2 b. α2δ2 c. α2γ2 d. α2ε2

Answer 50

a. α2β2 Hemoglobin (Hgb) A is characterized by pairs of α and β chains, α2β2. α2δ2 is Hgb A2, α2γ2 is Hgb F, and α2ε2 is Hgb Gower 2, an embryonic Hgb.

Question 51

Which of the following locations is not a site of extramedullary hematopoiesis? a. Bone marrow b. Liver c. Spleen d. Thymus

Answer 51

a. Bone marrow 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.

Question 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

Answer 52

b. Decreased production of erythropoietin Erythropoiesis is stimulated by erythropoietin, which is produced in the kidney, and renal failure can decrease the production of erythropoietin.

Question 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

Answer 53

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

Question 54

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 x 10^9/L, RBC count of 3.8 x 10^12/L, and platelet count of 225 x 10^9/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

Answer 54

a. A normal child 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.

Question 55

Which of the following cell types exhibit IgE receptors on their surface membranes? a. Basophils b. Eosinophils c. Band neutrophils d. Monocytes

Answer 55

a. Basophils 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.

Question 56

A 62-year-old female patient’s CBC showed the following results: total WBC count of 14.0 x 10^9/L, RBC count of 3.95 x 10^12/L, and platelet count of 245 x 10^9/L. The differential showed 65% segmented neutrophils, 10% bands, 15% lymphocytes, and 10% monocytes. Toxic granulation and Dohle 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

Answer 56

b. The patient has a bacterial infection 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. Dohle 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 Dohle bodies are not usually seen. If the patient had a parasitic infection, elevated numbers of neutrophils would be expected.

Question 57

A CBC on a patient with Chediak-Higashi syndrome is expected to exhibit which of the following? a. Giant platelets and Dohle-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

Answer 57

c. Giant fused granules and lysosomes in WBC cytoplasm 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 Dohle-like inclusions in the granulocytes is more characteristic of the May Hegglin anomaly, and large, dark granules are more associated with Alder-Reilly anomaly.

Question 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

Answer 58

a. Lymphocytosis, including increased variant/reactive lymphocytes 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.

Question 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

Answer 59

b. CD4 and CD8 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.

Question 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

Answer 60

c. Atypical chronic myelogenous leukemia 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.

Question 61

All of the following cells are derived from CFU-GEMM, common myeloid progenitor cells except: a. Basophils b. Lymphocytes c. Neutrophils d. RBCs

Answer 61

b. Lymphocytes Lymphocytes are derived from the common lymphocyte progenitor cell, whereas the other cells are derived from the common myeloid progenitor (or CFU-GEMM).

Question 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

Answer 62

c. Parasitic infection 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.

Question 63

Antibodies are produced by which of the following: a. Macrophages b. T lymphocytes c. Plasma cells d. Basophils

Answer 63

c. Plasma cells Antibodies are produced by lymphocytes, specifically B cells in the form of plasma cells. None of the other cells can produce antibodies.

Question 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

Answer 64

b. Chronic granulomatous disease (CGD) 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.

Question 65

A newly diagnosed patient has an acute leukemia. Which of the following would initially be the most useful in determining 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

Answer 65

c. Myeloperoxidase (MPO) and terminal dexoynucleotidyl transferase (TdT) 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, non-specific 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.

Question 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

Answer 66

a. Imatinib mesylate 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). All-trans 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.

Question 67

A 58-year-old female was seen by her physician for increasing fatigue. Her CBC shows the following results: WBC: 15.5 x 10^9/L RBC: 5.90 x 10^12/L Hgb: 17.5 g/dL Hct: 53.0% Platelet: 425 x 10^9/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

Answer 67

b. Polycythemia vera 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 x 10^9/L, and, additionally, white blood cell count >12 x 10^9 /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.

Question 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

Answer 68

b. Decreased erythropoietin levels 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.

Question 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)

Answer 69

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

Question 70

Which of the following is not classified as a myeloproliferative neoplasm? a. Chronic eosinophilic leukemia b. Essential thrombocythemia c. Mastocytosis d. Waldenstrom’s macroglobulinemia

Answer 70

d. Waldenstrom’s macroglobulinemia All are myeloproliferative neoplasms with the exception of Waldenstrom’s macroglobulinemia, which is a plasma cell disorder.

Question 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%

Answer 71

b. 15% 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.

Question 72

All of the following are considered to be signs of dyserythropoiesis except: a. Multinucleate RBCs b. Basophilic stippling c. Dohle bodies d. Oval macrocytes

Answer 72

c. Dohle bodies Dohle bodies are found in neutrophils. Multinucleate red blood cells (RBCs), nuclear bridging, basophilic stippling, siderotic granules, and macroovalocytes may be seen in dyserythropoietic RBCs.

Question 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

Answer 73

d. Siderotic granules 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.

Question 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

Answer 74

c. Megaloblastic anemia 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.

Question 75

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

Answer 75

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

Question 76

Whichof the followingisnot one of the recurrent genetic abnormalities seen in cases of acute myeloid leukemia? a. AML with t(8;21)(q22;q22); AML1(CBFα)/ETO b. AML with t(15;17)(q22;q12); (PML/RARα) c. AML with inv(16)/p(13;q22); (CBFβ /MYH11 d. AML with t(1;19)(q23;q13); (E2A/PBX1)

Answer 76

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

Question 77

AML with 11q23 (MLL) abnormalities are associated with which cell line? a. Eosinophil b. Erythrocyte c. Monocyte d. Neutrophil

Answer 77

c. Monocyte 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.

Question 78

T-cell ALL most commonly affects which of the following? a. Infants b. Teenaged males c. Adult females d. Elderly males

Answer 78

b. Teenaged males 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.

Question 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

Answer 79

c. Acute promyelocytic leukemia 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/RARα). The others currently do not have a specific recurrent cytogenetic abnormality and all are included in the “not otherwise classified” category.

Question 80

A 69-year-old female patient presented with symptoms of fatigue and easy bruising. A CBC was 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

Answer 80

a. AML with minimal differentiation 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.

Question 81

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 x 10^12/L RBC: 3.00 x 10^12/L Hgb 9.0 g/dL Hct 27.0% MCV 90 fL MCH 30 pg MCHC 33% Platelet 58 x 10^9/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

Answer 81

b. To look for leukemia cells in the spinal fluid 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.

Question 82

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: 58.5 x 10^12/L RBC: 3.90 x 10^12/L Hgb: 12.0 g/dL Hct: 36.0% MCV: 92 fL MCH: 3 pg MCHC: 33% Platelet: 132 x 10^9/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 Sezary syndrome b. The patient has developed prolymphocyticleukemia c. The patient has developed multiple myeloma d. The patient now has a concurrent case of CLL and ALL

Answer 82

b. The patient has developed prolymphocyticleukemia The patient most likely has Prolymphocytic leukemia (PLL), because prolymphocytes typically look like lymphocytes with a prominent nucleolus. Sezary syndrome has lymphoid cells with a convoluted nucleus, and plasma cells are the cells seen in multiple myeloma.

Question 83

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

Answer 83

d. Decreased immunoglobulin 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.

Question 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

Answer 84

b. Reed Sternberg cell 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.

Question 85

Which of the following appearances describes the types of cells seen in Sezary syndrome? a. Plasma cells containing immunoglobulin deposits b. Large circulating micromegakaryocytes c. Lymphocytes with convoluted, cerebriform nuclei d. Prolymphocytes with prominent azurophilic granules

Answer 85

c. Lymphocytes with convoluted, cerebriform nuclei Sezary cells are lymphoma cells characterized by cerebriform nuclei and irregular nuclear outlines.

Question 86

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

Answer 86

b. It results in the reduction of glutathione 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.

Question 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

Answer 87

a. Bone marrow response in producing more RBCs because of increased need 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.

Question 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

Answer 88

d. Diagnosis of suspected systemic fungal infection 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.

Question 89

A bone marrow sample 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

Answer 89

d. 10:1 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.

Question 90

Which of the following is not implicated as a cause of nonmegaloblastic macrocytic anemia? a. Alcoholism b. Hemochromatosis c. Hypothyroidism d. Liver disease

Answer 90

b. Hemochromatosis 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.

Question 91

Which of the following results is consistent with a diagnosis of aplastic anemia? a. Hypocellular bone marrow, absolute neutrophil count of 0.5 x 10^9/L, platelet count of 40 x 10^9/L, Hgb 8 g/dL b. Hypocellular bone marrow, absolute neutrophil count of 2.5 x 10^9/L, platelet count of 75 x 10^9/L, Hgb 10 g/dL c. Hypercellular bone marrow, absolute neutrophil count of 1.5 x 10^9/L, platelet count of 100 x 10^9/L, Hgb 14 g/dL d. Hypocellular bone marrow, absolute neutrophil count of 0.5 x 10^9/L, platelet count of 90 x 10^9/L, Hgb 11 g/dL

Answer 91

a. Hypocellular bone marrow, absolute neutrophil count of 0.5 x 10^9/L, platelet count of 40 x 10^9/L, Hgb 8 g/dL Diagnostic criteria for aplastic anemia include a hypocellular bone marrow, absolute neutrophil count less than 1.5 x 10^9/L, platelet count less than 50 x 10^9/L, and hemoglobin value less than 10 g/dL, with decreased reticulocyte response (Rodak, 2012).

Question 92

The following statement is true of mutations in α-thalassemia compared to those seen in β-thalassemia: a. Mutations in α-thalassemia occur as a result of reduced or absent expression of the globin gene b. Mutations in α-thalassemia occur as a result of the deletion of one or more globin genes c. The α-globin gene is expressed on chromosome 11 d. The β-globin gene is expressed on chromosome 16

Answer 92

b. Mutations in α-thalassemia occur as a result of the deletion of one or more globin genes Mutations in α-thalassemia occur as a result of deletions of one or more α-globin genes, and mutations in β thalassemia occur because of reduced or absent expression of one or more β-globin genes. The α-globin gene is found on chromosome 16, and the β globin gene is found on chromosome 11.

Question 93

A patient’s genotype is -α/-α. 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

Answer 93

d. Increased RBC count with microcytic/hypochromic RBCs This is a patient with α-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.

Question 94

Patients with sickle cell anemia and β-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. The mutations may not manifest clinically at birth because the presence of hemoglobin F decreases d. The mutations lead to elevations in α genes that compensate for the decreased gene expression

Answer 94

c. The mutations may not manifest clinically at birth because the presence of hemoglobin F decreases Hemoglobin (Hgb) F is present in larger quantities in fetal development and after a baby is born. As the baby makes more β-globin chains than γ-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.

Question 95

The thymus is a site used as a maturation compartment for: a. B cells b. T cells c. Megakaryocytes d. Monocytes

Answer 95

b. T cells The thymus is associated with lymphocyte development for precursor T cells, whereas the others all develop in the bone marrow.

Question 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 x 10^9/L b. 2.1 x 10^9/L c. 2.6 x 10^9/L d. 5.3 x 10^9/L

Answer 96

c. 2.6 x 10^9/L The equation used is number of cells counted/ (number of squares counted x area of square x depth of square) x reciprocal dilution to get cells/uL. Cells/uL x 10^6/uL /L will convert the answer to cells per liter. For this patient, 105/(8 x 1 mm^2 x 0.1 mm) x 20/1 = 2625 cells/uL, which converts to 2.6 x 10^9/L.

Question 97

Hereditary elliptocytosis results from defects in which of the following? a. Ankyrin b. Band 3 protein c. Spectrin d. Pyruvate

Answer 97

c. Spectrin 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.

Question 98

Primary neutrophil granules contain: a. Acetyltransferase, collagenase, gelatinase, lysozyme, β2-microglobulin b. Alkaline phosphatase, cytochrome b558, complement receptor 1, complement 1q receptor, vesicle-associated membrane-2 c. β2-Microglobulin, collagenase, gelatinase lactoferrin, neutrophil gelatinase-associated lipocalin d. Acid β-glycerophosphatase, cathespins, defensins, elastase, myeloperoxidase, proteinase-3

Answer 98

d. Acid β-glycerophosphatase, cathespins, defensins, elastase, myeloperoxidase, proteinase-3 Acid β-glycerophosphatase, cathespins, defensins, elastase, myeloperoxidase, and proteinase-3 are found in the primary granules of neutrophils. β2-Microglobulin, collagenase, gelatinase lactoferrin, and neutrophil gelatinase-associated lipocalin are found in the secondary (specific granules). Acetyltransferase, collagenase, gelatinase, lysozyme, β2-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.

Question 99

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 x 10^9/L RBC: 3.80 x 10^12/L Hgb: 12.0 g/dL Hct: 36.0% MCV: 95 fL MCH: 32 pg MCHC: 33% Platelet: 145 x 10^9/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

Answer 99

c. Refer the sample to the pathologist for further identification This sample, based on the intracellular red blood cell inclusions and boomerang shaped or banana-shaped 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.

Question 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

Answer 100

b. Donath-Landsteiner test 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.