Hematology MCQ

Hematology Questions and Answers
Mayo Clinic Internal Medicine Board

Review Questions and Answers
Edited by Robert D. Ficalora
Publisher: Oxford University Press Print Publication Date: Aug 2013

Print ISBN-13: 9780199985876 Published online: Nov 2013
DOI: 10.1093/med/ © Mayo Foundation for Medical
9780199985876.001.0001 Education and Research
Chapter: Hematology Questions and Answers
Author(s): Robert D. Ficalora
DOI: 10.1093/med/9780199985876.003.0009
Questions
Multiple Choice (choose the best answer)
Anemias and Myeloid Malignancies
1. A 67-year-old man is evaluated for exertional dyspnea. He recalls that 3

years ago he was told that he had anemia. In reviewing his records, you
note that at that time his hemoglobin level was 9.5 g/dL and his
hematocrit was 33% with an increased mean corpuscular volume (MCV);
the remainder of his complete blood cell count was normal. On physical
examination, he had conjunctival pallor, normal heart and lung findings,
no lymphadenopathy, no hepatomegaly or splenomegaly, and no petechiae
or ecchymoses. Diagnostic testing results are shown in Table 9.Q1.
Table 9.Q1
Component Finding
Page 1 of 19
Hemoglobin, g/dL 7.5
Hematocrit, % 23
Mean corpuscular volume, fL 110 (reference range, 86–98)
Leukocyte count, ×109/L 2.1

Neutrophils, % 20
Lymphocytes, % 70
Monocytes, % 6
Basophils, % 3
Eosinophils, % 1
Platelet count, ×109/L 64
Reticulocyte count, % of 0.3 (reference range, 0.5–1.5)

erythrocytes
Absolute reticulocyte count, 10.0 (reference range, 29.5–87.3)

×109/L
Peripheral blood film Dimorphic erythrocyte population

with pronounced macrocytes
Lactate dehydrogenase, U/L 150 (reference range, 140–280)
Which of the following is the most likely explanation for these findings?
a. Acute myeloid leukemia (AML)

b. Vitamin B12 deficiency
c. Hemolytic anemia
d. Myelodysplastic syndrome (MDS)
e. Primary myelofibrosis
2. A 45-year-old woman is admitted to the surgical service with severe

arterial insufficiency of the right second toe. She has no prior medical
history and takes no medications. Physical examination findings are
normal except for mild splenomegaly and signs of early gangrene in the
right second toe. All pulses are full and equal throughout. Diagnostic
testing results are shown in Table 9.Q2.
Table 9.Q2
Component Finding
Page 2 of 19
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Hematocrit, % 39

Segmented neutrophils, % 78
Band cells, % 4
Lymphocytes, % 20
Monocytes, % 5
Basophils, % 2
Eosinophils, % 1
Platelet count, ×109/L 1,300
Mean corpuscular volume, fL 88
Erythrocyte sedimentation 28
rate, mm/h
Leukocyte alkaline 110 (reference range, 13–130)

phosphatase score
Serum ferritin Within reference range
Serum iron Within reference range
Serum total iron-binding Within reference range

capacity
Peripheral blood film Increased large platelets with

some clustering; leukocytes and
erythrocytes are unremarkable
Bone marrow aspiration and Increased cellularity with

biopsy increased and atypical
megakaryocytes in clusters;
reticulin staining is normal
Chromosomal analysis Normal female karyotype (46XY)
Which of the following is the most likely diagnosis?
a. Essential thrombocythemia
b. Vasculitis
c. Philadelphia chromosome–negative chronic myeloid leukemia
(CML)
d. Primary myelofibrosis (PMF)
Page 3 of 19


3. A 22-year-old man is admitted to the hospital for an elective

cholecystectomy. You are asked to see him because he had anemia on
preoperative testing. He tells you that he has always been told by his
physicians that he has mild anemia; his medical history is otherwise
unremarkable. His vital signs are normal. His conjunctivae are mildly
icteric, and the spleen is palpable in the left upper quadrant. Findings on
the remainder of the physical examination are normal. Diagnostic testing
results are shown in Table 9.Q3.
Table 9.Q3
Component Finding
Hematocrit, % 34

Differential count Within reference ranges
Reticulocyte count, % 4
Absolute reticulocyte count, ×109/L 200 (reference range, 29.5–

87.3)
Peripheral blood film Polychromasia with numerous

microspherocytes
Which of the following tests would most likely help confirm the diagnosis?
a. Hemoglobin electrophoresis
b. Osmotic fragility test
c. Direct and indirect antiglobulin (Coombs) tests
d. Bone marrow aspiration and biopsy
4. A 28-year-old black man with sickle cell disease presents to the

emergency department with abdominal pain, chest pain, and shortness of
breath. His dyspnea evolved over 36 hours after a visit with his niece and
nephew. His history is significant for approximately 2 emergency
department visits or hospital admissions per year for painful crises. Three
years ago, he spent 4 weeks in the hospital after an episode of acute
chest syndrome. He has been taking hydroxyurea but only intermittently
because of financial concerns. His pulse is 116 beats per minute and
Page 4 of 19


regular, his blood pressure is 138/76 mm Hg, his respiratory rate is 18

breaths per minute, and his temperature is 38.3°C. Pulse oximetry shows
91% oxygen saturation with room air and 93% with 4 L of oxygen by nasal
cannula. His lungs have scattered inspiratory crackles in the right
midlung field. His spleen is not palpable. The remainder of the physical
examination findings are normal. Diagnostic testing results are shown in
Table 9.Q4.
Table 9.Q4
Component Finding
Hematocrit, % 25

Segmented neutrophils, % 70
Band cells, % 7
Lymphocytes, % 15
Monocytes, % 5
Basophils, % 2
Eosinophils, % 1
Creatinine, mg/dL 1.4
Peripheral blood film Anisopoikilocytosis with

multiple sickle cells
A chest radiograph shows a right middle and upper lobe air space
infiltrate. The patient is given supplemental oxygen, adequate pain
control, and intravenous antibiotics. Which of the following should you
now order?
a. Hydroxyurea
b. Erythrocyte exchange transfusion
c. Plasma exchange
d. Anticoagulation with unfractionated heparin
e. Aggressive intravenous fluid hydration
5. A 70-year-old man presents with weakness of his right arm and leg. His
symptoms began yesterday and are now resolved. He also reports a 6-
month history of recurrent headaches and fatigue. He is a nonsmoker. His
Page 5 of 19


medical history is significant for high blood pressure. His blood pressure
is 167/88 mm Hg, his oxygen saturation is 93% on room air, his face is
plethoric, and a right carotid bruit is heard. Other findings on physical
examination are normal. Diagnostic testing results are shown in Table
9.Q5.
Table 9.Q5
Component Finding
Hematocrit, % 58

Neutrophils, % 83
Lymphocytes, % 12
Monocytes, % 3
Basophils, % 2
Erythropoietin, mIU/mL <2 (reference range, 0–19)
Carotid ultrasonography shows a 30% stenotic lesion in the right carotid.

The patient is hospitalized and begins antiplatelet therapy. Which of the
following should you order next?
a. JAK2 V617F mutation testing

b. Fluorescence in situ hybridization (FISH) for BCR-ABL testing
c. Arterial blood gas analysis
d. Bone marrow aspiration and biopsy
6. A 42-year-old woman with a history of systemic lupus erythematosus

(SLE) presents with fatigue. She has been receiving anti–tumor necrosis
factor therapy and has been managing the SLE well. However, she has
recently experienced worsening fatigue. Her vital signs are normal. Her
face and conjunctivae are jaundiced, and she has a fading butterfly rash
on her face. The spleen is palpable on deep inspiration. Diagnostic testing
results are shown in Table 9.Q6, and the peripheral blood film is shown in
Figure 9.Q6.
Table 9.Q6
Page 6 of 19


Component Finding
Hematocrit, % 27

Differential count Within reference ranges
Reticulocyte count, % 4
Absolute reticulocyte count, ×109/L 170.0 (reference range, 29.5–

87.3)
Erythrocyte sedimentation rate, 25

mm/h
Lactate dehydrogenase, U/L 400 (reference range, 140–

280)
Total bilirubin, mg/dL 3.5 (reference range, 0.1–1.0)
Indirect bilirubin, mg/dL 3.0
Figure 9.Q6
Which of the following is the best interpretation of these data?
a. The hemolysis is predominantly intravascular.
Page 7 of 19


b. The bone marrow is not responding to the anemia.

c. Direct Coombs testing results should be positive.
d. Urine hemoglobin testing results should be positive.
7. A 58-year-old woman with active rheumatoid arthritis presents with

fatigue and joint pain. She received the diagnosis of rheumatoid arthritis
5 years earlier and has been taking prednisone 10 mg daily and
methotrexate with folate weekly. She has had chronic fatigue and anemia.
Her vital signs are normal. Her conjunctivae are pale, and she has active
synovitis affecting both knees, her wrists, and elbows, with rheumatoid
nodules on the extensor surface of her right forearm. The remainder of
the physical examination findings are normal. Diagnostic testing results
are shown in Table 9.Q7.
Table 9.Q7
Component Finding
Hematocrit, % 30

Neutrophils, % 90
Lymphocytes, % 8
Monocytes, % 2
Erythrocyte sedimentation rate, mm/h 50
Erythropoietin, mIU/mL 15 (reference range, 0–19)
Which of the following laboratory findings are consistent with this

condition?
a. Elevated hepcidin, elevated ferritin, elevated total iron-binding

capacity (TIBC), elevated serum iron
b. Elevated hepcidin, elevated ferritin, decreased TIBC, elevated
serum iron
c. Decreased hepcidin, elevated ferritin, decreased TIBC, elevated
serum iron
d. Elevated hepcidin, elevated ferritin, decreased TIBC, normal
serum iron
Page 8 of 19


e. Decreased hepcidin, elevated ferritin, elevated TIBC, normal

serum iron
Coagulation
8. A 62-year-old man underwent right total knee replacement 8 days ago.

Swelling has developed in his right lower extremity, and Doppler
ultrasonography confirms the presence of a right superficial femoral vein
thrombosis. His current medications include oxycodone and subcutaneous
unfractionated heparin. Results of preoperative tests, including a
complete blood cell count and liver and kidney function, were normal.
Other laboratory data include the following: hemoglobin 12.2 g/dL,
leukocyte count 8.5×109/L, and platelet count 60×109/L. In addition to
stopping the use of subcutaneous heparin, what is the next most
appropriate step in management of this patient?
a. Start low-molecular-weight heparin therapy.

b. Start intravenous therapeutic doses of heparin.
c. Start direct thrombin inhibitor therapy.
d. Start aspirin therapy.
9. A 45-year-old man presents with deep vein thrombosis of the right

femoral vein. Three months ago, he received a diagnosis of systemic lupus
erythematosus (SLE). In addition to confirming SLE, laboratory testing
also documented the presence of a lupus anticoagulant (LAC). There is no
family history of venous thrombosis. Current medications include
hydroxychloroquine. Laboratory testing shows normal results for a
complete blood cell count and for tests of liver and kidney function.
Special coagulation testing confirms the persistence of an LAC. What is
the most reasonable duration of warfarin anticoagulation for this patient?
a. 3 months
b. 1 year
c. 6 months
d. Long-term
e. 6 weeks
Page 9 of 19


10. A 20-year-old white woman has been admitted to the hospital with
pulmonary embolism. She has no chronic illnesses and is receiving no
medications except for combination estrogen-progesterone birth control
pills that she started using approximately 1 year earlier. Results were
normal for a complete blood cell count, baseline prothrombin time,
activated partial thromboplastin time (aPTT), and tests of kidney and liver
function. The patient is currently receiving therapeutic doses of
intravenous unfractionated heparin, and her aPTT is therapeutic at 72
seconds. A panel of thrombophilia tests has been performed. Which of the
following statements about her thrombophilia test results is correct?
a. DNA-based testing for factor V Leiden and prothrombin G20210A

mutations are reliable.
b. Low antithrombin confirms a hereditary deficiency state.
c. A positive result on lupus anticoagulant (LAC) testing confirms
antiphospholipid antibody syndrome.
d. Low protein S confirms the presence of a hereditary deficiency
state.
11. A 62-year-old man with chronic atrial fibrillation has been treated
with warfarin. He has no other chronic illnesses and is receiving no other
medications long-term except for lipid-lowering agents. Results of his
complete blood cell count and tests of renal and kidney function are
normal. He checks his prothrombin time monthly and has kept the
international normalized ratio (INR) within the therapeutic range (2–3)
for the duration of his therapy with warfarin. He has heard about recent
US Food and Drug Administration (FDA) approval of dabigatran, which
requires no monitoring, and he would like a prescription for this new
drug. Which of the following statements is true about the use of
dabigatran in atrial fibrillation compared with the well-managed use of
warfarin?
a. Switching to dabigatran would result in superior outcomes.

b. Switching to dabigatran would result in inferior outcomes.
c. Switching to dabigatran would provide no significant benefit.
d. Dabigatran is FDA approved for postoperative
thromboprophylaxis for knee and hip replacement surgery.
e. Dabigatran is FDA approved as an anticoagulant for patients who
have received a mechanical heart valve.
12. A 22-year-old woman is brought to the emergency department after

having 1 witnessed tonic-clonic seizure. She had appeared confused for
the preceding few hours. On examination, she is febrile and appears
slightly confused; otherwise, neurologic and physical examination
findings are normal. Laboratory testing results are shown in Table 9.Q12,
and the peripheral blood smear is shown in Figure 9.Q12.
Table 9.Q12
Page 10 of 19


Component Finding Reference Range
Hemoglobin, g/dL 8 12–15
Platelet count, ×109/L 50 150–450
Leukocyte count, ×109/L 8 3.5–10.0
Creatinine, mg/dL 2.5 0.8–1.3
Figure 9.Q12
What is the most appropriate next step in management?
a. Red blood cell transfusion

b. Platelet transfusion
c. Gamma globulin administration
d. Plasma exchange
13. A 72-year-old man with chronic atrial fibrillation has been receiving
dabigatran 75 mg twice daily for the past 6 months. He has not had any
thrombotic or hemorrhagic complications. He has a history of colon
polyps, for which he needs to undergo a colonoscopy with possible
polypectomy. Apart from an irregular pulse, his physical examination
findings are normal. Results were normal for a complete blood cell count
and tests of renal and liver function. The calculated creatinine clearance
is 28 mL/min. For how long should dabigatran use be discontinued before
the colonoscopy?
a. No need to discontinue
b. 24 hours
c. 48 hours
d. 3 days
e. 7 days
Page 11 of 19


Lymphoid Malignancies
14. At her annual physical examination, an asymptomatic 68-year-old

woman has lymphocytosis (32×109/L) with a normal hemoglobin level and
platelet count. On examination, she has 1-cm lymphadenopathy in the
cervical region and no palpable liver or spleen enlargement. A peripheral
blood smear shows identically appearing mature lymphocytes with
smudge cells. Flow cytometry of the peripheral blood lymphocytes shows
a monoclonal B population with dim expression of λ light chain and CD20
that is positive for expression of CD5, CD19, and CD23. Which of the
following is the best next step in her management?
a. Combination chemoimmunotherapy
b. Chlorambucil therapy
c. Allogeneic peripheral blood stem cell transplant
d. Combination monoclonal antibody therapy
e. Active monitoring for disease progression and complications
15. Ten years ago, a previously healthy 20-year-old woman presented to

her physician with a 2-month history of pruritis, drenching night sweats,
unintentional weight loss, and nonproductive cough. On examination, she
had 2-cm cervical lymphadenopathy. A computed tomographic scan
showed a 12-cm-diameter anterior mediastinal mass. An excisional biopsy
of a cervical lymph node showed nodular sclerosing Hodgkin lymphoma.
After she was treated with ABVD (doxorubicin [Adriamycin], bleomycin,
vinblastine, and dacarbazine) combination chemotherapy followed by
involved field radiotherapy, the disease was in complete remission. Now
you see her for the first time for an annual physical examination. The
disease remains in complete remission. Compared to her peers, this
patient is at increased risk of which of the following conditions?
a. Breast cancer
b. Coronary artery disease
c. Hypothyroidism
d. Skin cancer
e. All of the above
16. An 80-year-old man is admitted to the hospital after falling on an icy

sidewalk and fracturing his hip. He undergoes open reduction and
internal fixation of the fracture. At surgery, there does not appear to be
any bone disease at the fracture site. The patient was previously
asymptomatic. Physical examination findings are otherwise
unremarkable. Serum protein electrophoresis and immunofixation show
an IgM κ monoclonal protein (0.3 g/dL). The complete blood cell count
and serum creatinine levels are normal. Skeletal survey shows no
additional bone defects. Which of the following statements is true for this
patient?
a. He has multiple myeloma and requires treatment.
Page 12 of 19


b. He has a lower risk of a clinically significant lymphocytic or

plasma cell malignancy than patients with an IgG monoclonal
protein.
c. He requires a radioisotope bone scan to evaluate his bone
integrity.
d. He requires regular follow-up and serial measurements of his
monoclonal protein level.
e. He has a 10% annual risk of multiple myeloma.
17. A 75-year-old African American man was seen last week by his
primary care physician for mild dyspnea. He has also noted intermittent
peripheral edema. During the evaluation, an electrocardiogram showed
low-voltage QRS complexes in the limb leads. The troponin T level was
elevated (0.07 ng/mL). This finding suggested the need for a coronary
angiogram, which showed no significant coronary artery disease. An
echocardiogram showed diffuse left ventricular thickening with a
granular texture to the myocardium and a septal thickness of 2.5 cm
(normal <1.1 cm). The complete blood cell count results were normal.
Serum and urine protein electrophoresis and immunofixation were
unremarkable. Serum free light chain levels were not increased. What is
the most likely diagnosis?
a. AA amyloidosis
b. Light chain–related amyloidosis
c. Hypertrophic obstructive cardiomyopathy
d. Amyloidosis due to transthyretin deposition
e. Amyloidosis due to β 2-microglobulin deposition
18. A 55-year-old man presented to his primary care physician for

evaluation of fatigue. He was previously healthy with the exception of
chronic musculoskeletal low back pain, for which he occasionally takes
nonsteroidal anti-inflammatory drugs. On examination, he is pale.
Complete blood cell count results are as follows: hemoglobin 8.3 g/dL,
mean corpuscular volume 73 fL, leukocyte count 6.9×109/L, and platelet
count 398×109/L. Results of the fecal occult blood test are positive.
During upper and lower endoscopy, a 1.2×2.5-cm ulcerative lesion is
noted in the lesser curvature of the stomach. The lesion is biopsied and
identified as a MALT lymphoma. Which of the following is characteristic
of MALT lymphoma?
a. Most cases are treated with anthracycline-based chemotherapy.

b. It is caused by chronic stimulation with Chlamydophila psittaci.
c. Radiotherapy is necessary in most cases.
d. It frequently undergoes transformation to a large-cell lymphoma.
e. The combination of amoxicillin, omeprazole, and clarithromycin is
the most appropriate first-line treatment.
Page 13 of 19


19. A 73-year-old woman presented to the emergency department with

new-onset back pain, confusion, and constipation over the past week. Her
past medical history is significant only for hypertension. On examination,
she is slightly pale with slow cognition and point tenderness over the
lumbar spine. Plain films of the lumbar spine show osteolytic lesions in
L2, L3, and L5. Laboratory values are as follows: hemoglobin 9.3 g/dL,
leukocyte count 4.6×109/L with a normal differential count, platelet count
230×109/L, creatinine 1.6 mg/dL, total calcium 13.1 mg/dL, albumin 3.6
g/dL, and total protein 9.1 g/dL. What is the most likely diagnosis?
a. Metastatic breast cancer

b. Hydrochlorothiazide use
c. Multiple myeloma
d. Primary hyperparathyroidism
e. Milk alkali syndrome
20. A 48-year-old man presents to the emergency department with a 6-

week history of progressively worsening abdominal pain and night
sweats. Physical examination findings were significant for palpable
bilateral 2-cm axillary lymph nodes and diffuse abdominal tenderness
with no rebound or guarding. Computed tomography of the abdomen and
pelvis showed retroperitoneal and mesenteric lymphadenopathy.
Excisional biopsy of an axillary node was positive for diffuse, large B-cell
lymphoma. Positron emission tomography showed fluorodeoxyglucose-
avidity in the axillary, mesenteric, and retroperitoneal lymph nodes.
Results of the bone marrow examination were normal. Which of the
following is the best next step?
a. Combination therapy with rituximab, cyclophosphamide,

doxorubicin, vincristine, and prednisone (R-CHOP)
b. Observation
c. Combination therapy with cyclophosphamide, doxorubicin,
vincristine, and prednisone (CHOP)
d. Autologous stem cell transplant
e. Involved field radiotherapy
Answers
1. Answer d.
MDS most commonly manifests as isolated macrocytic anemia. MDS can

evolve to include pancytopenia over several years; the typical peripheral
smear findings include a dimorphic erythrocyte population (microcytes
and oval macrocytes) with an overall prominent macrocytosis and an
MCV around 110 fL. The chronicity of MDS—in particular, anemia
preceding the diagnosis of pancytopenia by several years—is in contrast
to the typically acute manifestation of AML, which is therefore an unlikely
possibility in this patient. Primary myelofibrosis, a myeloproliferative
neoplasm, causes fibrosis in the bone marrow, resulting in extramedullary
Page 14 of 19


hematopoiesis and significant splenomegaly, and typically does not cause

a macrocytic anemia. Vitamin B12 deficiency can cause a megaloblastic
anemia and manifest with slowly evolving macrocytic anemia and
eventually pancytopenia, but the peripheral smear would not show a
dimorphic erythrocyte population. (See Tefferi and Vardiman in the
“Suggested Reading” list.)
2. Answer a.
Extreme thrombocytosis may be reactive and occur with severe iron

deficiency or inflammatory states (with elevated erythrocyte
sedimentation rates) or after splenectomy; patients are typically
asymptomatic. Clonal thrombocytosis is related to a myeloproliferative
neoplasm, which usually causes splenomegaly. Typical bone marrow
findings include a hypercellular bone marrow with increased atypical
megakaryocytes in clusters. Essential thrombocythemia may cause
extreme thrombocytosis (platelet count >1,000×109/L); however, it can
also occur less commonly with polycythemia rubra vera (typically with
erythrocytosis), the cellular phase of PMF, or rarely CML. The normal
karyotype makes CML much less likely since it typically manifests with
the Philadelphia chromosome t(9;22). Increased reticulin fibrosis would
have been seen on the bone marrow biopsy if the patient had PMF. (See
Tefferi in the “Suggested Reading” list.)
3. Answer b.
When a patient presents with premature gallstones, one should consider

whether they may be due to pigment gallstones from chronic hemolysis
causing indirect hyperbilirubinemia. The presence of microspherocytes is
consistent with hereditary spherocytosis, and the diagnostic test is an
osmotic fragility test, which identifies a congenital membrane defect.
Typically, acquired warm autoimmune hemolytic anemia, which produces
positive Coombs test results, can cause spherocytes as well; however, the
history of lifelong anemia makes this diagnosis unlikely. A hemoglobin
electrophoresis would help in diagnosing thalassemia or a
hemoglobinopathy; however, these conditions do not manifest with
microspherocytes on the peripheral blood film. There is no indication for
a bone marrow biopsy since the reticulocyte response is appropriate and
no other cytopenias are apparent. (See Gallagher in the “Suggested
Reading” list.)
4. Answer b.
The patient has acute chest syndrome, a sickle cell anemia complication
that is an indication for urgent red cell (not plasma) exchange transfusion
to decrease the hemoglobin S level to less than 30% to 35%. Gentle fluid
resuscitation is appropriate (along with oxygen support and antibiotics,
since about one-third of acute chest syndrome events are initiated by or
associated with bacterial pneumonia). Aggressive fluid resuscitation,
leading to overhydration, might cause pulmonary edema and worsen the
Page 15 of 19


oxygenation. Pulmonary embolism is possible, but full anticoagulation is

not warranted until embolism is documented. Use of hydroxyurea might
have prevented this crisis, but it is of no value for the acute condition.
(See Vij and Machado in the “Suggested Reading” list.)
5. Answer a.
Polycythemia may be secondary, as with erythropoietin- mediated causes

such as chronic hypoxemia, living at high altitude, and high oxygen
affinity hemoglobinopathies. Polycythemia vera is a myeloproliferative
neoplasm that can manifest with arterial thrombosis secondary to
hyperviscosity from the increased concentration of erythrocytes. The low
erythropoietin rules out erythropoietin-mediated causes, leaving the
presumptive diagnosis of polycythemia vera. With JAK2 V617F mutation
testing of peripheral blood, results are positive for approximately 90% of
patients who have polycythemia vera. FISH for BCR-ABL testing would
screen for chronic myeloid leukemia, which does not manifest with
polycythemia. Although bone marrow aspiration and biopsy would be
helpful, it is not immediately necessary and could be considered later.
(See Patnaik and Tefferi in the “Suggested Reading” list.)
6. Answer c.
Hematologic complications of SLE include anemia of chronic disease,

pure red cell aplasia, and warm autoimmune hemolytic anemia (WAIHA).
The presentation and laboratory data suggest hemolysis, and the blood
smear shows spherocytes. These findings are consistent with WAIHA,
which causes extravascular hemolysis. The reticulocytosis suggests that
the bone marrow response is adequate. In intravascular hemolysis, the
urine is positive for hemoglobin. (See Packman in the “Suggested
Reading” list.)
7. Answer d.
Rheumatoid arthritis is a chronic inflammatory disorder that may lead to

anemia of chronic disease. Anemia of chronic disease results from the
effect of elevated cytokines on hematopoiesis, including upregulation of
hepcidin, leading to increased ferritin from iron malutilization and
downregulation of ferroportin, the main iron exporting system.
Transferrin is also downregulated, leading to decreased TIBC and normal
to decreased serum iron levels. (See Weiss and Goodnough in the
“Suggested Reading” list.)
8. Answer c.
The timing and degree of thrombocytopenia are consistent with immune-

mediated heparin-induced thrombocytopenia type II. Unfractioned
heparin and low-molecular-weight heparin are contraindicated. Aspirin
would not be the sole management agent for established thrombosis. The
most appropriate step is to start a direct thrombin inhibitor.
Page 16 of 19


9. Answer d.
Presentation with a vascular thrombosis and persistence of a LAC for 12

weeks or more satisfies the criteria for an antiphospholipid syndrome.
This patient has a high risk for recurrent venous thrombosis on
discontinuing anticoagulation; thus, long-term warfarin is recommended
with periodic reassessment for safety.
10. Answer a.
DNA-based testing is reliable for patients receiving heparin or warfarin

and for patients who have acute thrombosis. However, acute thrombosis
and heparin can cause lower antithrombin activity results, which should
be verified at another time, when heparin and acute thrombosis are not
factors. A single positive test result for LAC does not confirm
antiphospholipid syndrome; follow-up testing at 12-week intervals is
required to demonstrate persistence of LAC. Acute thrombosis and
estrogen use can lower protein S levels; thus, abnormally low results
require follow-up confirmation.
11. Answer c.
Among patients randomly assigned to receive dabigatran, overall

outcomes were noninferior when compared with the well-managed use of
warfarin, thus providing no significant advantages. The group of patients
that derived the most benefit from dabigatran was the group with INRs
outside the recommended therapeutic range. Dabigatran is FDA approved
only to reduce the risk of stroke and systemic embolism in patients with
nonvalvular atrial fibrillation.
12. Answer d.
Plasma exchange is the treatment of choice for thrombotic

thrombocytopenic purpura (TTP). Although red blood cell transfusion may
be indicated, it does not address the underlying pathogenesis of TTP.
Platelets are thought to be contraindicated in TTP because of the
theoretical possibility of worsening the TTP. Gamma globulin is ineffective
in increasing the platelet count in TTP.
13. Answer d.
Dabigatran is cleared through the kidneys. It has a prolonged half-life in

patients who have a creatinine clearance less than 30 mL/min compared
with patients who have a creatinine clearance greater than 30 mL/min.
14. Answer e.
Chronic lymphocytic leukemia (CLL) is a clonal lymphoproliferative

disorder of mature lymphocytes. The clinical diagnosis requires a B-
lymphocyte count of more than 5×109/L. Peripheral blood smears
typically show smudge cells, which are lymphocytes that have broken
during processing of the slide. The clinical course of CLL is chronic in
Page 17 of 19


most patients. For those with early-stage disease, standard practice is to

withhold treatment until the disease is active or progressive. However,
patients need to be monitored for disease progression, autoimmune
complications, infections, and second cancers.
15. Answer e.
Hodgkin lymphoma therapy is curative in about 80% of cases. However,

there are late complications of therapy, particularly in those treated
before modern chemotherapy and radiotherapy. At 15 years, the risk of
death from other causes surpasses that of risk of death from Hodgkin
lymphoma. Patients are at higher risk of secondary malignancies,
cardiovascular disease, thyroid disorders, and infertility than the general
population. Many of these conditions can be attributed to chemotherapy
and radiotherapy.
16. Answer d.
This patient has monoclonal gammopathy of undetermined significance

(MGUS), the most common dysproteinemia. In MGUS, the M protein level
is typically less than 3 g/dL, the bone marrow has less than 10% plasma
cells, and the hemoglobin, creatinine, calcium, and bone radiographs are
normal. The risk of progression to a lymphocytic or plasma cell
malignancy is about 1% per year. Patients with an IgM or IgA monoclonal
protein are at higher risk of progression than those with an IgG protein.
Patients with MGUS need to be observed.
17. Answer d.
The patient has senile cardiac amyloidosis. This syndrome is usually

isolated to the heart with few clinically significant deposits elsewhere,
and the echocardiographic findings are often out of proportion to the
degree of symptoms. Transthyretin is the protein causing the amyloid
deposits; most patients have wild-type transthyretin.
18. Answer e.
With combination antibiotic therapy, 70% of gastric MALT lymphomas are

cured. In cases refractory to antibiotics, tumors may carry the t(11;18)
translocation, and involved field radiotherapy is effective. Combination
chemotherapy is reserved for advanced disease. The majority of cases are
associated with Helicobacter pylori infection.
19. Answer c.
This patient has multiple myeloma with evidence of end-organ damage

from the plasma cell proliferative disorder (hypercalcemia, renal failure,
anemia, and osteolytic bone lesions). The other answer choices are
possible causes of hypercalcemia, but only multiple myeloma accounts for
all the presenting symptoms, including the elevated level of total protein.
20. Answer a.
Page 18 of 19


This patient has advanced-stage, diffuse, large B-cell lymphoma, and R-

CHOP chemotherapy is the standard of care. Rituximab is an anti-CD20
monoclonal antibody that improves overall survival when added to CHOP
chemotherapy for aggressive B-cell lymphomas. For patients whose
disease relapses or is refractory, autologous stem cell transplant is the
standard therapy. Radiotherapy can be used in combination with
chemotherapy in early-stage (I-IIA) nonbulky disease but is not standard
therapy for advanced disease.
Suggested Reading
Gallagher PG. Red cell membrane disorders. Hematology Am Soc
Hematol Educ Program. 2005:13–8.
Packman CH. Hemolytic anemia due to warm autoantibodies. Blood Rev.

2008 Jan;22(1):17–31. Epub 2007 Sep 27.
Patnaik MM, Tefferi A. The complete evaluation of erythrocytosis:

congenital and acquired. Leukemia. 2009 May;23(5):834–44. Epub 2009
Mar 19.
Tefferi A. Annual clinical updates in hematological malignancies: a

continuing medical education series: polycythemia vera and essential
thrombocythemia: 2011 update on diagnosis, risk-stratification, and
management. Am J Hematol. 2011 Mar;86(3):292–301.
Tefferi A, Vardiman JW. Myelodysplastic syndromes. N Engl J Med. 2009

Nov 5;361(19):1872–85.
Vij R, Machado RF. Pulmonary complications of hemoglobinopathies.

Chest. 2010 Oct;138(4):973–83.
Weiss G, Goodnough LT. Anemia of chronic disease. N Engl J Med. 2005

Mar 10;352(10):1011–23.
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Hematology Questions and Answers

Mayo Clinic Internal Medicine Board

Publisher: Oxford University Press Print Publication Date: Aug 2013

Hematology Questions and Answers

Chapter: Hematology Questions and Answers

Author(s): Robert D. Ficalora

Multiple Choice (choose the best answer)

Anemias and Myeloid Malignancies

1. A 67-year-old man is evaluated for exertional dyspnea. He recalls that 3

Hemoglobin, g/dL 7.5

Mean corpuscular volume, fL 110 (reference range, 86–98)

Leukocyte count, ×109/L 2.1

Platelet count, ×109/L 64

Reticulocyte count, % of 0.3 (reference range, 0.5–1.5)

Absolute reticulocyte count, 10.0 (reference range, 29.5–87.3)

Peripheral blood film Dimorphic erythrocyte population

Lactate dehydrogenase, U/L 150 (reference range, 140–280)

a. Acute myeloid leukemia (AML)

2. A 45-year-old woman is admitted to the surgical service with severe

Hemoglobin, g/dL 13.2

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford

date: 24 August 2020

Leukocyte count, ×109/L 15.5

Platelet count, ×109/L 1,300

Mean corpuscular volume, fL 88

Leukocyte alkaline 110 (reference range, 13–130)

Serum ferritin Within reference range

Serum iron Within reference range

Serum total iron-binding Within reference range

Peripheral blood film Increased large platelets with

Bone marrow aspiration and Increased cellularity with

Chromosomal analysis Normal female karyotype (46XY)

Which of the following is the most likely diagnosis?

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford

date: 24 August 2020

3. A 22-year-old man is admitted to the hospital for an elective

Hemoglobin, g/dL 11.2

Leukocyte count, ×109/L 9.0

Platelet count, ×109/L 295

Mean corpuscular volume, fL 89

Absolute reticulocyte count, ×109/L 200 (reference range, 29.5–

Peripheral blood film Polychromasia with numerous

4. A 28-year-old black man with sickle cell disease presents to the

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford

date: 24 August 2020

regular, his blood pressure is 138/76 mm Hg, his respiratory rate is 18

Hemoglobin, g/dL 7.9

Mean corpuscular volume, fL 80

Leukocyte count, ×109/L 16.0

Platelet count, ×109/L 490

Creatinine, mg/dL 1.4

Peripheral blood film Anisopoikilocytosis with

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford

date: 24 August 2020

Hemoglobin, g/dL 20.5

Mean corpuscular volume, fL 88

Leukocyte count, ×109/L 12.5

Platelet count, ×109/L 600

Erythropoietin, mIU/mL <2 (reference range, 0–19)