Hematology study guide

1. A 33-year-old female presents with complaints of dyspnea on exer on, fa gue, and malaise. On
examina on, you note that she exhibits conjunc val pallor. In addi on, her ngernails exhibit
the appearance as shown in the image.

Which one of the following is most useful currently?

A. Serum folate
B. Direct an globulin test
C. Serum iron
D. Complete blood count
E. Serum B12
Answer: D
What is the next value that should be considered?
A. MCV
B. MCH
C. MCHC
D. RDW
E. Red cell mass
Answer: A
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 The pa ent’s MCV is 70 fL (80-100 fL).
What is this called?
A. Microcy c
B. Normocy c
C. Macrocy c
Answer: A

What is the next test to order in the workup of this pa ent?

A. C-reac ve protein
B. Whole blood lead level
C. Homocysteine
D. Methylmalonic acid
E. Iron studies
Answer: E
Which one of the following set of ndings are most likely in this pa ent?
A. Serum iron low, serum ferri n elevated, TIBC low
B. Serum iron low, serum ferri n low, TIBC low
C. Serum iron low, serum ferri n low, TIBC high
D. Serum iron high, serum ferri n high, TIBC high
E. Serum iron high, serum ferri n low, TIBC high
Answer: C
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 2. A 54-year-old postmenopausal female with a long history of severe rheumatoid arthri s,
presents with complaints of worsening fa gue and decreased exercise tolerance. Fecal occult
blood test is nega ve.
Labs:
Hb 10.1 g/dL (14-16 g/dL)
MCV 78 fL (80-100 fL)

Based on these ndings, you diagnose the pa ent with a microcy c anemia and decide to order
iron studies. Which one of the following results are most likely?
A. Serum iron low, serum ferri n high, TIBC low
B. Serum iron low, serum ferri n low, TIBC low
C. Serum iron low, serum ferri n low, TIBC high
D. Serum iron high, serum ferri n high, TIBC high
E. Serum iron high, serum ferri n low, TIBC high

Answer: A
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 3. A 3-year-old boy develops mental status changes and abdominal pain.
Labs reveal the following:
Hb 8.8 g/dL (13 – 15 g/dL)
MCV 75 fL (80 – 100 fL)
Peripheral blood smear reveals:

Which one of the following studies should be ordered?

A. Hemoglobin electrophoresis
B. Bone marrow biopsy
C. Serum folate
D. Serum methylmalonic acid
E. Whole blood lead

Answer: E
 4. A 26-year-old male graduate student develops petechiae, fa gue, and a fever. A CBC reveals the
following:
Hb 7.7 g/dL (14 – 16 g/dL)
MCV 90 fL (80 – 100 fL)
WBC 1,200/µL (4,400 – 11,000/µL)
Platelet count 7,000/µL (150,000 – 400,000/µL)
A bone marrow biopsy reveals the following:

Which one of the following disorders will explain this pa ent’s condi on?
A. Folate de ciency
B. B12 de ciency
C. Aplas c anemia
D. Primary myelo brosis
E. Metasta c carcinoma involving the bone marrow
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 Answer: C

5. Due to inconsolable crying, a previously well 6-month-old African American boy is seen in the
emergency department. No family history is available as he was adopted at birth. On
examina on, he withdraws his hands and feet to touch. A CBC reveals the child to be anemic.
The peripheral blood smear shows the following.

Of the following, which is the most likely to provide diagnos c informa on?
A. Hemoglobin electrophoresis
B. Bone marrow biopsy
C. Serum folate
D. Serum methylmalonic acid
E. Whole blood lead
Answer: A
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 6. A 30-year-old female presents to her physician with complaints of postprandial RUQ pain and
nausea. Exam reveals RUQ tenderness and an abdominal ultrasound con rms the presence of
gallstones. Following cholecystectomy, the following specimen is received in the lab.

What does the image suggest?

A. Iron de ciency
B. Excess dietary oxalate
C. Hepa c dysfunc on
D. Impaired absorp on of bile salts
E. Chronic hemolysis
Answer: E

7. A 24-year-old male presents for a pre-employment physical exam. He reports having chronic
fa gue. CBC reveals a normocy c anemia with elevated MCHC. Re culocyte count is elevated. A
peripheral blood smear reveals the following:
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 Which one of the following condi ons do you suspect?
A. Disseminated intravascular coagula on (DIC)
B. Hemoly c uremic syndrome (HUS)
C. Thrombo c thrombocytopenic purpura (TTP)
D. Beta thalassemia major
E. Hereditary spherocytosis
Answer: E
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 8. A 33-year-old female with a history of systemic lupus erythematosus (SLE) presents to the
physician’s o ce with complaints of decreased exercise tolerance and fa gue. The exam reveals
mild scleral icterus and moderate splenic enlargement. A CBC reveals a moderately severe
normocy c anemia. The pa ent has the following peripheral blood smear ndings.

Which one of the following tests should be performed next?

A. Osmo c fragility
B. Bone marrow biopsy
C. Flow cytometry
D. Direct an globulin test
E. Hemoglobin electrophoresis
Answer: D
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 9. While on a medical mission trip to Africa, a 23-year-old medical student of Asian descent
develops severe fa gue. Examina on reveals scleral icterus. Labs reveal a normocy c anemia
with elevated serum levels of lactate dehydrogenase (LDH). Which one of the following
mechanisms is the most likely cause of this presumed hemolysis?
a. Autoimmune
b. Oxida ve injury
c. Mechanical trauma
d. Lack of complement inhibi on
e. Inherited red cell membrane defect
Answer: B

10. A 63-year-old female with a history of autoimmune thyroidi s, presents with complaints of
fa gue and impaired sensa on in the extremi es. A CBC reveals a macrocy c anemia with Hb
9.7 g/dL (13 – 15.5 g/dL) and MCV of 120 fL (80 – 100 fL). Peripheral blood smear shows the
following (see image). Serum homocysteine and methylmalonic acid are both increased.
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 Which one of the following de ciencies do you suspect?
a. Iron
b. Folic acid
c. Thiamine
d. Cobalamin
e. Niacin
Answer: D
11. A 13-year-old boy with sickle cell disease presents with complaints of worsening fa gue,
decreased exercise tolerance, and joint aches (arthralgia). Labs reveal worsening of his anemia
compared to his baseline with a re culocyte produc on index of 0.1%. Which one of the
following is the most likely e ology of his worsening anemia?
a. Acute GI blood loss
b. Iron de ciency
c. Inadequate erythropoie n release
d. Pernicious anemia
e. Viral infec on
Answer: E

12. A 2-year-old boy was brought to the emergency department by his mother for oropharyngeal
bleeding following a fall nearly 6 hours ago. His mother reports that the pa ent tends to ooze
blood from immuniza on sites but denies history of bruising or hematoma forma on. The
pa ent was on an bio cs for a recent ear infec on. There was no known family history of a
bleeding disorder. On exam, the pa ent is alert, in no apparent distress, and his development is
appropriate for age. Two small lacera ons are noted on the inner lower lip, each of which are
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 oozing blood. Remainder of exam within normal limits (no petechiae, ecchymoses, or joint
swelling). Laboratory nding are as follows:
Hemoglobin: 12.3 g/dl (10.5-13.5)
Hematocrit: 35.4% (33.0-39.0)
WBC: 7,900/microL (6,000-17,500)
Platelets: 368,000/microL (150-400)
PT: 11.3 s (10.0-12.8)
PTT: 37.2 s (24.4-33.2)

Which one of the following is the most likely e ology for this pa ent’s bleeding?
a. Hemophilia A
b. Hemophilia B
c. Von Willebrand disease
d. Factor VIII inhibitor
e. Factors VII de ciency

Answer: C

13. A 2-year-old male presents to his pediatrician because of a “limp” of two days’ dura on. The
child was adopted and thus no family history is available. On exam, the right knee is edematous,
erythematous, and tender. Arthrocentesis (joint aspira on) of the a ected joint reveals frank
blood. Laboratory studies are as follows:
PT: normal
PTT: prolonged (corrects with mixing study)
Platelet count: normal
Plasma von Willebrand factor an gen (VWF:Ag): normal

Which one of the following is most likely?

a. Accidental inges on of warfarin
b. Bernard-Soulier syndrome
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 c. Glanzmann thrombasthenia
d. Hemophilia A
e. Hemophilia B
Answer: D

14. A 58-year-old male with a history of renal failure was admi ed with complaints of hemorrhoidal
bleeding. Labs, as shown below revealed prolonga on of PT. The pa ent was then transfused
with fresh frozen plasma (FFP) to correct his coagulopathy and stop the bleeding. In addi on, he
was transfused with 4 units packed red blood cells (pRBCs) for his severe anemia. During the
transfusion, the pa ent became hypertensive and dyspneic requiring intuba on. Exam revealed
crackles and jugular venous distension. The transfusion was terminated, and a transfusion
reac on workup was ini ated. He responded well to diuresis with furosemide and was
extubated the following morning.

ADMISSION LABORATORY VALUES:

Hb 6.5 mg/dL
Hct 19.2 %
Platelet 156,000/microL
WBC 14,200/microL
PT 18 seconds (10-13)
APTT 32 seconds (24-33)
BUN 98 mg/dL
Cr 2.8 mg/dL

INVESTIGATION OF REPORTED TRANSFUSION REACTION

PRELIMINARY INVESTIGATION: Direct AGT Plasma HGB Urine
Pre reac on specimen Neg Neg N/A
Post reac on specimen - Neg Neg Neg

Specimen ABO Rh Ab Screen

Pre specimen Type O cells Rh neg Neg
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 Post specimen Type O cells Rh neg Neg

What is the most likely explana on?

A. Acute hemoly c transfusion reac on
B. Allergic transfusion reac on
C. Febrile transfusion reac on
D. Transfusion associated cardiac overload (TACO)
E. Transfusion related acute lung injury (TRALI)
Answer: D

15. A 55-year-old male was admi ed with an intes nal obstruc on. The following results were
recorded by the blood bank technologist:

Forward Grouping Reverse Grouping Rh Tes ng

An -A An -B A Cells B Cells An -DD
4 0 0 4+ 3+

What is the pa ent’s blood type?

A. A +
B. B +
C. AB -
D. O +
Answer: A
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16. A 54-year-old female presents to the Emergency Department with abdominal cramping and
lethargy over the last few days. She also reports her stools have been black and s cky. Her PMH
is remarkable for a history of pep c ulcer disease (PUD) resul ng in GI bleeding. She was
transfused with 2 units packed RBCs 2 months ago for the same symptoms.
CBC results are shown below.
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 The pa ent was admi ed to the hospital. The pa ent is type A pos with a nega ve an body
screen. Because the pa ent was ac vely bleeding, 4 units of packed RBCs (PRBCs) were
crossmatched and transfused.

Two days later, the pa ent was discharged, with orders to follow up with her GI doctor for
further tes ng and treatment. Three days a er discharge she s ll felt weak and returned to the
ER. On examina on, it was noted that the pa ent’s eyes and skin appeared jaundiced. The
pa ent had a fever of 100F. Repeat lab results are shown below.

What has happened?

A. Acute hemoly c transfusion reac on
B. Delayed hemoly c transfusion reac on
C. Febrile transfusion reac on
D. Transfusion related infec on
E. Transfusion related acute lung injury
Answer: B
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 17. A 19-year-old male presents the physician with complaints of malaise, fa gue, and sore throat.
On examina on, the pa ent is febrile with prominent cervical lymphadenopathy, a palpable
spleen, and mild RUQ tenderness. Lymphocytosis is noted. A peripheral blood smear reveals the
following:

Which one of the following organisms is the most likely cause of this man’s condi on?
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 A. Rubella
B. Cytomegalovirus
C. Epstein-Barr virus
D. Rhinovirus
E. West Nile virus
Answer: C
18. A 67-year-old female with renal cell carcinoma presents to her physician with complaints of
confusion and blurry vision. A CBC reveals a hematocrit of 53% (39-45%). She does not smoke
and has no history of lung disease. Her oxygen satura on is 99% on ambient air. MRI of the head
is nega ve for metasta c disease. Her blood smear shows no teardrop cells, nucleated red cells,
or immature white cells. Which one of the following do you suspect?
a. Metasta c disease to the bone marrow
b. Ectopic erythropoie n secre on
c. Autoimmune-mediated s mula on of erythropoiesis
d. Extramedullary hematopoiesis
e. Chemotherapy e ect
Answer: B

19. A 65-year-old man with a history of pancrea c carcinoma develops erythema, swelling, and pain
in his right calf. Which one of the following tests should be performed?
a. MRI
b. CT
c. Ultrasound
d. Angiogram
e. X-ray
Answer: C
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 20. A 70-year-old Black male presents to the emergency department with a worsening cough. On
examina on, he is noted to have dullness to percussion over the right lower lobe along with a
posi ve ‘e’ to ‘a’ sound. Chest x-ray con rms the presence of pneumonia. Labs show a
neutrophilic leukocytosis with a le shi , hypercalcemia, an elevated total serum protein, and
decreased serum albumin. Peripheral blood smear shows the following:

Which one of the following is a risk factor for the development of this condi on?
A. Radia on
B. Asbestos
C. Chronic sun exposure
D. Cigare e smoking
E. Alcohol inges on
Answer: A
This pa ent most likely has mul ple myeloma (MM).
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 Which one of the following tests should be performed at this me?
A. Flow cytometry on peripheral blood
B. Serum parathyroid hormone (PTH) level
C. Cytogene cs
D. Serum protein electrophoresis
E. Liver biopsy
Answer: D

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 Note the “punched-out”
ly c bone lesions.

21. A 60-year-old female presents to her physician complaining of malaise. Examina on reveals the
pa ent to have lost 10 pounds since her last visit. Marked leukocytosis is noted on the CBC. A
peripheral smear is shown. The leukocyte alkaline phosphatase score is low. Which one of the
following disorders do you suspect?
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 a. Bacterial infec on
b. Essen al thrombocythemia
c. Polycythemia vera
d. Chronic myelogenous leukemia
e. Acute lymphoblas c leukemia

Answer: D
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 22. A 70-year-old male presents to his physician’s o ce for his annual health maintenance
examina on (which is unremarkable). Later that week, his physician receives a call from the
pathologist sta ng that the CBC and blood smear are sugges ve of chronic lymphocy c leukemia
(CLL). What addi onal nding on the peripheral blood smear was likely seen?
a. Howell-Jolly bodies
b. Heinz bodies
c. Teardrop-shaped erythrocytes
d. Basophilia
e. Smudge cells
Answer: E
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 23. A 3-year-old girl presents with recurrent bruising, fa gue, and a low-grade fever. A CBC reveals
neutropenia, anemia, and thrombocytopenia. Numerous blasts, which are posi ve for TdT, are
noted on the blood smear. What is the diagnosis?
a. Acute lymphoblas c leukemia
b. Acute myelogenous leukemia
c. Chronic lymphocy c leukemia
d. Chronic myelogenous leukemia
e. Infec ous mononucleosis
Answer: A
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 24. A 60-year-old male presents to the physician with complaints of severe pruritus, par cularly
a er bathing. He also reports episodes of blurry vision. CBC reveals elevated hematocrit and
platelet count. His oxygen satura on and erythropoie n levels are normal. Which one of the
following disorders is most likely?
a. Chronic myelogenous leukemia
b. Polycythemia vera
c. Essen al thrombocythemia
d. Primary myelo brosis
e. Dehydra on
Answer: B

25. An 18-month-old boy presents with recurrent bacterial infec ons. It is determined that he has
an immune de ciency due to a muta on in the BTK gene. Which one of the following is
expected?
a. Increased incidence of ataxia
b. Recurrent eczematous skin rash
c. Delayed separa on of the umbilical cord
d. Leukocytosis
e. Decreased levels of immunoglobulins
Answer: E

26. A 30-year-old female with a history of recurrent spontaneous abor ons (i.e., miscarriages)
develops acute shortness of breath and dyspnea. On examina on, her le calf is erythematous
and edematous. Following recovery from her acute event, which one of the following is the most
likely e ology of her condi on?
a. Muta on in factor V
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 b. An phospholipid an body
c. Prothrombin 20210 muta on
d. De ciency of protein C
e. De ciency of an thrombin
Answer: B

27. A 34-year-old male with a long-history of HIV develops severe shortness of breath with oxygen
satura on of 78% on room air. The CXR is shown. This pa ent’s pneumonia is most likely due to
which one of the following organisms.

A. Mycobacterium tuberculosis
B. Staphylococcus aureaus
C. Pseudomonas aeruginosa
D. Pneumocys s jiroveci
E. Histoplasma capsulatum
Answer D
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 PCP treatment:
1. Trimethoprim-sulfamethoxazole (TMP-SMX)
2. Adjunc ve glucocor coids are o en given as well.

28. A 40-year-old female presents with a nonspeci c febrile illness with generalized
lymphadenopathy. She is concerned about having contracted HIV. Though she denies the use of
illicit drugs, she recently began a sexual rela onship with a new partner. Which one of the
following is the most common route of HIV transmission worldwide?
a. Heterosexual contact
b. Homosexual contact
c. Blood transfusion
d. Occupa onal needle s ck injury
e. IV drug abuse
Answer: A

29. A 35-year-old man presents with severe fa gue and recurrent bleeding from the nose and gums.
A CBC reveals pancytopenia. A peripheral blood smear reveals the following nding.
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 Which one of the following disorders do you suspect?
a. Bacterial infec on
b. Essen al thrombocythemia
c. Polycythemia vera
d. Chronic myelogenous leukemia
e. Acute promyelocy c leukemia

Answer: E

30. A 2-month-old infant presents because the mother no ced that the umbilical cord stump is s ll
present. She recalls that the umbilical cord had already separated by this me in her other
children. On examina on, there is erythema and swelling around the umbilicus and residual cord
segment. A rash is noted around the mouth as well. Which one of the following is most likely?
a. Decreased expression of selec ns
b. Impaired isotype switching
c. Muta on in the BTK gene
d. Adenosine deaminase de ciency
e. Decreased expression of CD18
Answer: E
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 31. A 2-year-old male presents to the pediatrician with complaints of recurrent sinus and ear
infec ons. His history is remarkable for prolonged bleeding following circumcision. On
examina on, the child exhibits petechiae and purpura as well as an eczematous rash. His platelet
count is 20,000/mm3. Which one of the following is most likely?
a. Decreased expression of selec ns
b. Impaired isotype switching
c. Muta on in the WAS gene
d. Adenosine deaminase de ciency
e. Decreased expression of CD18
Answer: C

32. A 2-year-old pa ent presents with recurrent derma s and other infec ons by catalase-posi ve
bacteria. A lymph node biopsy reveals areas of granulomatous in amma on. Which one of the
following is most likely?
a. Defec ve NADPH oxidase
b. Myeloperoxidase de ciency
c. Muta on in BTK gene
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 d. Impaired isotype switching
e. Human immunode ciency virus infec on
Answer: A

33. A pa ent with a history of recurrent viral infec ons is noted to have a lack of a thymic shadow
on chest x-ray. Laboratory studies reveal hypocalcemia. Which one of the following is most
likely?
a. Decreased CD18 expression
b. NADPH oxidase de ciency
c. Impaired isotype switching
d. Selec ve IgA de ciency
e. 22q11 dele on
Answer: E

34. A 20-year-old male su ers splenic rupture in a motor vehicle accident and undergoes emergency
splenectomy. This pa ent is now at an increased risk of which of the following?
a. Recurrent Candida infec ons
b. Streptococcal pneumoniae sepsis
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 c. Disseminated Neisseria infec on
d. Recurrent Staphylococcal abscesses
e. Severe diarrhea due to Giardia lamblia

Answer: B
Pa ents such as this, who have undergone splenectomy, might exhibit which of the following on their
peripheral blood smear.
A. Bite cells
B. Heinz bodies
C. Howell-Jolly bodies
D. Hypersegmented neutrophils
E. Spherocytes
Answer: C
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Figure 159-2, Goldman-Cecil Textbook of Medicine, 26th edi on.
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 35. A 3-month-old boy has had recurrent infec ons since birth, including Candida albicans,
Pneumocys s jiroveci, Pseudomonas aeruginosa, rotavirus, and cytomegalovirus. Despite
treatment with an bio cs and an fungal drugs, he dies at 6 months of age. At autopsy, lymph
nodes are small with very few lymphocytes and no germinal centers. The thymus, Peyer patches,
and tonsils are hypoplas c. There is a family history of other males with similar ndings. Which
of the following best describes the abnormality that led to this pa ent’s demise?
A. De ciency of CD4 cells due to congenital HIV infec on
B. Dele on involving chromosome 22q11
C. Muta on in the common γ chain of cytokine receptors
D. Muta on in the Bruton tyrosine kinase (BTK) gene
E. Muta on in CD40 ligand
Answer: C

36. An 18-year-old college student presents to his physician with palpable inguinal
lymphadenopathy. He has been experiencing a fever with drenching night sweats on at least 4
nights each week for the past month and has lost 7 kg. On exam, he has palpable abnormal
lymph nodes in the le neck, le axilla, and bilateral inguinal/femoral areas. An excisional biopsy
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 of a le supraclavicular fossa lymph node demonstrates sca ered cells like that shown in the
image.

What is the most likely diagnosis?

A. Burki lymphoma
B. Follicular lymphoma
C. Hodgkin lymphoma
D. Large B-cell lymphoma
E. Small lymphocy c lymphoma

Answer: C

37. A 23-year-old man has no ced bilateral lower neck mass lesions. These lesions, which are rm,
have not resolved a er a trial of oral an bio cs. An excisional biopsy of an involved node reveals
predominantly small lymphocytes and granulocytes, small numbers of eosinophils, and
occasional monoclonal large mul nucleated cells that are posi ve for CD15 and CD30. Further
workup is likely to reveal a history of infec on by which of the following?
A. EBV
B. HCV
C. HHV-8
D. HPV
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 E. HTLV-1

Answer: A

At what stage of disease is this pa ent if further workup con rms tumor involving only the cervical
lymph nodes?
A. I
B. II
C. III
D. IV

Answer: A
MODIFIED ANN ARBOR STAGING SYSTEM FOR HODGKIN LYMPHOMA

Table 177-3, Goldman-Cecil Textbook of Medicine, 26th edi on.

38. A 55-year-old female presents with back pain. Workup reveals the following:
Hb: 9.1 g/dL (13 – 15.5 g/dL)
MCV: 79 fL (80 – 100 fL)
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 WBC: 3.8 mm3 (4,400 – 11,000/mm3)
Platelet: 400K/mm3 (150 – 400K/mm3)

Peripheral blood smear reveals nucleated red blood cells, immature myeloid precursors
(promyelocytes, myelocytes, and metamyelocytes), and tear-drop shaped red cells.

These ndings are most consistent with which one of the following.
A. Aplas c anemia
B. Extramedullary hematopoiesis
C. Leukemoid reac on
D. Leukoerythroblastosis
E. Pancytopenia

Answer: D
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The bone marrow biopsy shows the presence of an abnormal cell popula on within the marrow. Further
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 Index

1. Iron de ciency anemia

2. Anemia of chronic disease
3. Sideroblas c anemia
4. Aplas c anemia
5. Sickle cell anemia
6. Hemoly c anemia
7. Hemoly c anemia
8. G6PD de ciency
9. Pernicious anemia
10. Parvovirus (aplas c crisis)
11. Von Willebrand disease
12. Hemophilia A
13. Transfusion reac on (TACO)
14. Blood typing
15. Transfusion reac on (delayed HTR)
16. Infec ous mononucleosis
17. Erythrocytosis
18. Venous thromboembolism
19. Mul ple myeloma
20. Chronic myelogenous leukemia
21. Chronic lymphocy c leukemia
22. Acute lymphoblas c leukemia
23. Polycythemia vera
24. Bruton Agammaglobulinemia
25. An -phospholipid an body syndrome
26. Acquired immunode ciency syndrome (AIDS) with Pneumocys s jirovecii pneumonia
27. Human immunode ciency virus (HIV)
28. Acute promyelocy c leukemia
29. Leukocyte adhesion de ciency
30. Wisko -Aldrich syndrome
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ti
 31. Chronic granulomatous disease
32. DiGeorge syndrome
33. Splenic dysfunc on
34. Severe combined immunode ciency syndrome (SCID)
35. Hodgkin lymphoma
36. Hodgkin lymphoma
37. Metasta c tumor within bone marrow
ti
ti
fi