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WebPath Hematopathology Questions ANSWERED

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					Question 1

A 61-year-old man has had dull, constant back pain for 3 months. He recently
developed a cough productive of yellowish sputum. On physical examination there are
crackles at the right lung base. A plain film radiograph of the spine reveals several 1 to
2 cm lytic lesions of the vertebral bodies. Laboratory studies show sodium 140 mmol/L,
potassium 4.4 mmol/L, chloride 101 mmol/L, CO2 26 mmol/L, glucose 78 mg/dL, urea
nitrogen 49 mg/dL, creatinine 5 mg/dL, total protein 8.3 g/dL, albumin 3.7 g/dL,
alkaline phosphatase 176 U/L, AST 45 U/L, ALT 22 U/L, and total bilirubin 1.2 mg/dL.
A sputum culture grows Streptococcus pneumoniae. Which of the following pathologic
findings is most likely to be seen in a bone marrow biopsy from this man?


A      Scattered small granulomas


B     Nodules of small mature lymphocytes


C     Occasional Reed-Sternberg cells


D      Numerous plasma cells (D) CORRECT. The findings suggest multiple myeloma.
He has a markedly increased level of serum globulins. The renal failure and the
increased risk for encapsulated bacterial infections is typical


E     Hypercellularity with many blasts

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Question 2

A 33-year-old woman has experienced low grade fevers, night sweats, and generalized
malaise for the past 2 months. On physical examination she has non-tender cervical and
supraclavicular lymphadenopathy. A cervical lymph node biopsy is performed. On
microscopic examination at high magnification there are occasional Reed-Sternberg
cells along with large and small lymphocytes and bands of fibrosis. Which of the
following is the most likely diagnosis?


A      Burkitt lymphoma


B       Hodgkin lymphoma (B) CORRECT. Reed-Sternberg cells are multinucleated
with large nucleoli. Variants of them called lacunar cells are also seen with some forms
of Hodgkin lymphoma.


C     Cat scratch disease
D      Mycosis fungoides


E     Multiple myeloma

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Question 3

A 17-year-old adolescent has had malaise for the past 3 weeks. He has a mild
pharyngitis on physical examination, as well as tender axillary and inguinal
lymphadenopathy. A CBC shows Hgb 14.0 g/dL, Hct 42.2%, MCV 90 fL, platelet
count 301,300/microliter, and WBC count 8120/microliter with "atypical lymphocytes"
on the peripheral blood smear. His illness is most likely to be acquired via which of the
following mechanisms?


A      From a genetic abnormality


B     While on a date (B) CORRECT. Infectious mononucleosis with Epstein-Barr virus
(EBV) infection is typically acquired with close personal contact.


C     As a result of an insect bite


D      Through an environmental exposure at work


E     Without any known etiology

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Question 4

A 42-year-old man has had fevers for the past 4 weeks. On physical examination his
temperature is 37.8 C. Laboratory studies show a Hgb of 12.2 g/dL, Hct 37.1%, MCV
92 fL, platelet count 243,000/uL, and WBC count 75,000/uL. The WBC differential
count shows 82 segs, 8 bands, 3 metamyelocytes, 1 myelocyte, 4 lymphocytes, and 1
monocyte. The leukocyte alkaline phosphatase (LAP) score is high at 130. Which of the
following laboratory test findings is most likely to be present in this man?


A      Bone marrow karyotype of 46, XY, t(9;22)
B     Serologic titer of 1:1024 for anti-double stranded DNA


C     Serum vitamin B12 level of 100 pg/mL


D      4+ ketonuria and 4+ proteinuria


E      Blood culture positive for Streptococcus, viridans group (E) CORRECT. The
high WBC count with left shift (but no blasts) and the high LAP score are consistent with
a leukemoid reaction. The term 'leukemoid' is used because there is a markedly
increased WBC count, with immature forms of WBCs, similar to leukemia.

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Question 5

A clinical study is conducted involving adults from 18 to 80 years of age who
underwent splenectomy for blunt force abdominal trauma. An age-matched control
group of patients consists of patients who have congestive splenomegaly. The
laboratory findings from these subjects are analyzed. Which of the following laboratory
test findings is most likely to be observed in the study group following splenectomy?


A      Thrombocytopenia


B      RBC Howell-Jolly bodies (B) CORRECT. Increased numbers of red blood cell
inclusions such as nuclear fragments (Howell-Jolly bodies) and degenerated
hemoglobin (Heinz bodies) appear following splenectomy.


C     Decreased RBC distribution width


D      Leukopenia


E     Nucleated RBCs

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Question 6

A 30-year-old man gives a history of a sore throat with fever followed by 6 weeks of
malaise. On physical examination he has mildly tender generalized lymphadenopathy.
A cervical lymph node biopsy is performed and on microscopic examination shows
prominent germinal centers in follicles with a diffuse polyclonal hyperplasia composed
of lymphocytes, plasma cells, and macrophages. Which of the following is the most
likely diagnosis?


A      Lymphocytic lymphoma


B     Hodgkin lymphoma


C       Infectious mononucleosis (C) CORRECT. This is a typical history for infectious
mononucleosis. The peripheral blood usually demonstrates atypical lymphocytosis.
Liver involvement may lead to mild icterus.


D      Human immunodeficiency virus infection


E     Brucellosis

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Question 7

A 30-year-old man has had a progressively worsening productive cough for one month.
On physical examination, a few small non-tender lymph nodes are palpable in the
axillae, and the tip of the spleen is palpable. Laboratory studies show Hgb 10.2 g/dl, Hct
31.1%, MCV 90 fL, WBC count 67,000/microliter, and platelet count 36,000/microliter.
Microscopic examination of his peripheral blood smear shows many blasts with Auer
rods. Which of the following is the most likely diagnosis?


A      Leukemoid reaction


B      Acute myelogenous leukemia (B) CORRECT. The high WBC count with the
blasts and Auer rods are very characteristic for an acute myelogenous leukemia
AUER=ACUTE!


C     Chronic lymphocytic leukemia


D      Acute lymphoblastic leukemia


E     Leukoerythroblastosis

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Question 8

A 68-year-old man has had malaise for over a year. On physical examination, there are
no abnormal findings. A CBC shows: Hgb 10.5 g/dL, Hct 31.5%, MCV 85 fL, platelet
count 211,000/microliter, and WBC count 6980/microliter. A bone marrow biopsy is
performed and microscopic examination shows that maturation is occurring in all cell
lines and there are no abnormal cells seen. Stainable iron in the bone marrow is
increased. He has a total serum iron of 130 microgm/dL and total iron binding capacity
(TIBC) of 230 microgm/dL. Which of the following underlying diseases is he most
likely to have?


A      Diverticulosis


B       Hepatitis C infection (B) CORRECT. He has findings of anemia of chronic
disease, with a high % iron saturation and increased iron stores. Hepatitis C viral
infection is a chronic disease.


C     Systemic lupus erythematosus


D      Atrophic gastritis


E     Fanconi anemia

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Question 9

A 68-year-old man has noted the presence of several lumps on the right side of his neck
for the past 5 months. On physical examination he has firm, non-tender, movable lymph
nodes palpable in right posterior cervical region. He does not have splenomegaly or
hepatomegaly. Laboratory studies show Hgb 11.3 g/dL, Hct 40%, MCV 88 fL, platelet
count 256,000/microliter, and WBC count 7230/microliter. A cervical lymph node
biopsy is performed and on microscopic examination shows numerous crowded follicles
composed of small, monomorphic lymphocytes. Which of the following is the most
likely diagnosis?


A      Chronic lymphocytic leukemia


B     Poorly differentiated lymphocytic lymphoma (B) CORRECT. Lymphadenopathy
with malignant lymphoma is typically nontender, as contrasted with the
lymphadenopathy of infections.


C     Infectious mononucleosis


D     Hodgkin lymphoma, lymphocyte predominance type


E     Reactive hyperplasia

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Question 10

A 45-year-old man has had increasing abdominal discomfort with abdominal
enlargement for the past two years. On physical examination, the spleen can be felt
below the left costal margin. There is no fluid wave. An abdominal CT scan reveals
massive (estimated 3000 gm size) splenomegaly. Which of the following underlying
conditions is he most likely to have?


A      Myelofibrosis A) CORRECT. Myeloproliferative disorders, and myelofibrosis in
particular, are known to cause massive splenomegaly.


B     Sickle cell anemia


C     Portal hypertension


D     Infectious mononucleosis


E     Hemochromatosis

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Question 11

A 72-year-old Caucasian man has had increasing fatigue for the past year. On physical
examination there are no abnormal findings. Laboratory studies show a Hgb of 9.1
gm/dL, Hct 27.9%, MCV 96 fL, WBC count 3700/microliter, and platelet count
125,000/microliter. The WBC differential count on the peripheral blood smear shows
53 segs, 5 bands, 2 metamyelocytes, 1 myelocyte, 32 lymphs, 7 monos, and 5 nucleated
RBCs/100 WBCs. He has a negative direct and indirect Coombs test. Which of the
following diseases is he most likely to have?


A      Metastatic carcinoma (A) CORRECT. Metastatic tumor involving marrow or
marrow fibrosis reduces normal hematopoiesis leads to a peripheral
'leukoerythroblastic' picture with immature RBC's and WBC's in the peripheral blood
smear.


B     Chronic alcoholism


C     Malabsorption


D      Hemoglobinopathy


E     Chronic blood loss


F     Systemic lupus erythematosus

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Question 12

A 40-year-old woman has had a week long course of fever and mental confusion.
Physical examination shows T 38.2 C, P 100/minute, RR 20/minute, and BP 100/60 mm
Hg. She has widespread petechiae of skin and mucosal surfaces. Laboratory studies
show her serum urea nitrogen is 52 mg/dL with creatinine 5.3 mg/dL. She has a
hemoglobin of 12.2 g/dL, hematocrit 36.8%, MCV 93 fL, platelet count
19,000/microliter, and WBC count 8180/microliter. Schistocytes are seen on her
peripheral blood smear. Her condition deteriorates rapidly following platelet
transfusion. At autopsy, pink hyaline thrombi are found in small myocardial arteries.
Which of the following is the most likely diagnosis?


A      Disseminated intravascular coagulopathy


B     Idiopathic thrombocytopenic purpura


C       Thrombotic thrombocytopenic purpura (C) CORRECT. The hyaline thrombi are
typical for TTP, as is the pentad of fever, mental changes, renal failure,
thrombocytopenia, and microangiopathic hemolytic anemia. Platelet transfusion is
contraindicated.
D     Trousseau syndrome


E     Warm autoimmune hemolytic anemia

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Question 13

For the past 4 months, a 62-year-old man has noted increasing fatigue and shortness of
breath with minimal exercise. He has noted some abdominal discomfort over the past
month. He has been healthy all his life. On physical examination he has non-tender
cervical lymphadenopathy. The liver span is 10 cm in the right mid-clavicular line; the
edge is smooth and palpable just below right costal margin. The spleen is palpated 3 cm
below left costal margin on inspiration. A CBC shows WBC count 23,100/microliter
with 16 segs, 2 bands, 78 lymphs, and 4 monos, Hgb 11.9 g/dL, Hct 36%, MCV 90, and
platelet count 277,300/microliter. The direct Coombs test is positive. Which of the
following is the most likely diagnosis?


A     Leukemoid reaction


B     Chronic myelogenous leukemia


C     Acute myelogenous leukemia


D     Acute lymphocytic leukemia


E      Chronic lymphocytic leukemia (E) CORRECT. Most of the circulating cells are
small, mature lymphocytes with CLL. Most persons with CLL are older adults.


F     Systemic lupus erythematosus

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Question 14

A 16-year-old boy has had a low energy level for as long as he can remember. On
physical examination he has a palpable spleen tip. A CBC shows Hgb of 8.8 g/dL, Hct
24.1%, MCV 65 fL, platelet count 187,000/microliter, and WBC count 7400/microliter.
His serum ferritin is 3740 ng/mL. A bone marrow biopsy is performed and on
microscopic examination reveals a myeloid:erythroid ratio of 1:4, and there is 4+
stainable iron. Which of the following is the most likely diagnosis?


A      G6PD deficiency


B      Beta-thalassemia (B) CORRECT. Reduced beta-globin chain synthesis from
beta-thalassemia leads to RBC microcytosis, hypochromia, ineffective erythropoiesis,
and excessive iron absorption.


C     Sickle cell anemia


D      Hereditary spherocytosis


E     Malaria

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Question 15

An 83-year-old woman fell and broke her left wrist. She underwent open reduction with
internal fixation of the radial head fracture. Postoperatively she received heparin
prophylaxis for thromboembolism and diuretics to treat pulmonary edema. Laboratory
studies showed:

On admission: Hgb 13.2 g/dL, Hct 39.1%, MCV 85 fL, platelet count
209,000/microliter, WBC count 5720/microliter, prothrombin time 13 seconds, partial
thromboplastin time 28 seconds

1 week later: Hgb 13 g/dL, Hct 38.8%, MCV 86 fL, platelet count 101,000/microliter,
WBC count 6310/microliter, prothrombin time 12 seconds, partial thromboplastin time
27 seconds

She suddenly developed difficulty in moving her right arm. What is the most likely
cause for her findings?


A      Hypersplenism


B     Idiopathic thrombocytopenic purpura


C     Disseminated intravascular coagulation


D      Drug-induced thrombocytopenia (D) CORRECT. In about 5% of patients
receiving heparin, antibodies develop to a complex of platelet factor 4 with heparin, and
in 5 to 14 days there is a marked drop in platelet count. The feared complication is
thrombosis ('white clot' syndrome), which can be arterial or venous, and in this patient a
thrombotic stroke is likely to have occurred.


E     Thrombotic-thrombocytopenic purpura

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Question 16

A 48-year-old man has experienced increasing malaise and difficulty concentrating at
work for the past 6 months. On physical examination he has splenomegaly but no
lymphadenopathy. He is afebrile. Laboratory studies show Hgb 12.0 g/dL, Hct 35.8%,
MCV 92 fL, platelet count 390,000/uL, and WBC count 190,000/uL with differential
count 73 segs, 12 bands, 6 metamyelocytes, 2 myelocytes, 2 myeloblasts, and 5 lymphs.
The leukocyte alkaline phosphatase (LAP) score is only 8. A bone marrow biopsy is
performed. Which of the following microscopic findings is most likely to be found in
this biopsy?


A      Sheets of plasma cells


B      Atypical cytokeratin positive glands


C      Numerous mature and immature myeloid cells (C) CORRECT. He has chronic
myelogenous leukemia (CML) which is a form of myeloproliferative process in which
one or more cell lines (myeloid, erythroid, megakaryocytic) becomes increased. Unlike
acute myelogenous leukemia, blasts are uncommon in CML


D      A decrease in all cell lines


E     Granulomas that have many acid fast bacilli

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Question 17

A 30-year-old woman has the sudden onset of fever, abdominal pain, tachycardia, and
nausea. On physical examination her vital signs include T 37.6 C, P 90/minute, RR
18/minute, and BP 100/60 mm Hg. Her conjunctivae are icteric. The spleen tip is
palpable. Laboratory studies shows Hgb 9.0 g/dL, Hct 27.3%, MCV 99 fL, platelet
count 209,500/microliter, and WBC count 6840/microliter. Her reticulocyte count is
0.1%. On microscopic examination of her peripheral blood smear, the RBC's are small
and lack central pallor. Which of the following most likely initiated this woman's acute
illness?


A      Quinacrine use


B       Parvovirus infection (B) CORRECT. The findings point to hereditary
spherocytosis. Parvovirus B19 infects erythroid precursors and can lead to an aplastic
crisis in persons with hemoglobinopathies.


C     Decreased oxygen tension


D      Exposure to cold


E     Transfusion therapy

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Question 18

A 35-year-old man has had fatigue, fever, and episodes of epistaxis for the past 3
months. On physical examination his temperature is 37.4 C. Laboratory studies show
Hgb 12.5 g/dL, Hct 37.6%, MCV 89 fL, platelet count 170,000/microliter, and WBC
count 52,000/microliter. Examination of his peripheral blood smear shows large blasts
with Auer rods. Which of the following is the most likely diagnosis?


A      Chronic myelogenous leukemia


B     Infectious mononucleosis


C     Plasma cell leukemia


D      Chronic lymphocytic leukemia


E      Acute myelogenous leukemia (E) CORRECT. Auer rods are formed of the
cytoplasmic granules of the myeloid blasts of AML and are a typical finding with AML

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Question 19
A 42-year-old woman has noticed during the past month that even minor bumps
produce major bruises over her body. On physical examination she has areas of purpura
on the skin of her arms and legs. She is afebrile. Laboratory studies shows her
prothrombin time is 12.9 seconds (control 13 sec) and partial thromboplastin time 26.2
seconds (control 25 sec). Her CBC shows a Hgb of 11.1 g/dL, Hct 33.1%, MCV 84 fL,
platelet count 790,000/uL, and WBC count 45,400/uL. A bone marrow biopsy is
performed and on microscopic examination shows hypercellularity with myeloid and
megakaryocytic hyperplasia. Which of the following is the most likely diagnosis?


A      Epstein-Barr virus infection


B      Myeloproliferative disorder (B) CORRECT. There is an increase in numbers of
more than one cell line in the marrow. Paradoxically, there is bleeding because the
platelets are functionally abnormal, even though there are lots of them.


C      Drug reaction to recent antibiotic therapy


D      Wiskott-Aldrich syndrome


E      Megaloblastic anemia

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Question 20

A clinical study is performed with subjects who are adults found to have anemia. Their
clinical histories and laboratory findings are reviewed. It is observed that ingestion of a
drug preceded development of the anemia in some of the subjects, but not in others.
Which of the following conditions is most likely to be found in persons without a
history of drug ingestion?


A      G6PD deficiency


B      Autoimmune hemolytic anemia


C      Macrocytic anemia


D      Aplastic anemia


E      Microcytic anemia (E) CORRECT. Microcytic anemia is usually the result of an
iron deficient state not associated with drug usage.

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Question 21

A 25-year-old African-American man is given anti-malarial prophylaxis for a trip to
West Africa. Over the next week he develops increasing fatigue. On physical
examination there are no abnormal findings. Laboratory studies show a hematocrit of
30%. Examination of his peripheral blood smear shows red blood cells with numerous
Heinz bodies. There is a family history of this disorder, with males, but not females,
affected. Which of the following is the most likely diagnosis?


A      Beta-thalassemia


B     Sickle cell anemia


C     Alpha-thalassemia


D      Hereditary spherocytosis


E     G6PD deficiency (E) CORRECT. He has glucose-6-phosphate dehydrogenase
(G6PD) deficiency, which can result in a hemolytic anemia on exposure to oxidizing
agents such as certain drugs such as antimalarials. This is an X-linked disorder. The
Heinz bodies within the RBCs are formed from denatured hemoglobin.

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Question 22

An autopsy study is conducted to determine what changes in the size of the spleen take
place with hematologic disorders. The spleen weights are recorded for adult patients
who died from complications of their hematologic disease. For which of the following
diseases is the spleen most likely to remain normal in size?


A      Hemolytic anemia


B     Alcohol abuse
C      Myelofibrosis


D       Idiopathic thrombocytopenic purpura (D) CORRECT. Though with ITP there are
circulating antibodies to platelets that lead to platelet destruction, the spleen itself is
usually not enlarged.


E     Sickle cell anemia

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Question 23

A 51-year-old man has become increasingly fatigued for the past 10 months. On
physical examination there are no abnormal findings. Laboratory studies show his Hgb
is 9.2, Hct 27.9%, MCV 132 fL, platelet count 242,000/microliter, and WBC count
7590/microliter. Which of the following morphologic findings is most likely to be
present on examination of his peripheral blood smear?


A     Hypersegmented neutrophils (A) CORRECT. The increased MCV points to a
macrocytic anemia such as a megaloblastic anemia that can also have
hypersegmented PMN's from reduced numbers of nuclear divisions.


B      Nucleated red blood cells


C      Blasts


D      Hypochromic, microcytic RBC's


E     Schistocytes

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Question 24

A 72-year-old man has been feeling tired for the past 8 months. On physical
examination there are no abnormal findings. Laboratory studies show Hgb 10.4 g/dL,
Hct 30.3%, MCV 72 fL, platelet count 239,000/uL, and WBC count 7500/uL with
automated differential count of 70.1% grans, 18.8% lymphs, and 11.1% monos. His
total bilirubin is 1.0 mg/dL. Which of the following morphologic findings is most likely
to be seen on his peripheral blood smear?
A     Fragmentation


B     Many nucleated forms


C     Hypochromasia (C) CORRECT. The most probable cause of a hypochromic,
microcytic anemia in an older male is iron deficiency anemia.


D     Spherocytosis


E     Howell-Jolly bodies

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Question 25

A 19 year old primigravida of Southeast Asian ancestry gives birth at 35 weeks
gestation a male infant. On physical examination the infant is markedly hydropic.
Laboratory studies show his hematocrit is 17% and the peripheral blood smear reveals
numerous nucleated red blood cells and even a few erythroblasts. The red blood cells
display marked anisocytosis and poikilocytosis. Which of the following diseases is most
likely to be present in this infant?


A     Sickle cell anemia


B      Alpha-thalassemia (B) CORRECT. The form of alpha-thalassemia seen in
Southeast Asia has two abnormal alpha globin genes on the same chromosome, so it is
possible to inherit two bad sets, leading to a complete lack of alpha globin chain
formation, so that fetal hemoglobin cannot be made, and there is a severe anemia.


C     Hemoglobin E disease


D     G6PD deficiency


E     Hereditary elliptocytosis

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Question 26
A 45-year-old man has had worsening arthritis and swelling of his feet for the past year.
On physical examination he has rales audible in all lung fields. A chest radiograph
shows cardiomegaly and pulmonary edema. Laboratory studies show Hgb 13.0 g/dL,
Hct 39.1%, MCV 86 fL, platelet count 255,500/uL, and WBC count 5920/uL. His
serum iron is 406 microgram/mL with iron binding capacity 440 microgram/mL and
ferritin 3830 ng/mL. Which of the following is the most likely diagnosis?


A      Beta-thalassemia


B     Autoimmune hemolytic anemia


C     Anemia of chronic disease


D      Polycythemia vera


E     Pernicious anemia


F      Hereditary hemochromatosis F) CORRECT. Hereditary hemochromatosis results
from increased iron absorbtion with markedly increased iron stores. The iron
accumulation in tissues results in manifestations such as hepatomegaly, skin
pigmentation, diabetes mellitus, heart disease, arthritis, and hypogonadism.

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Question 27

A 50-year-old man has had a fever with cough for a month. On physical examination
his temperature is 37.5 C. There are crackles auscultated in upper lung fields. A chest
radiograph shows a reticulonodular pattern with upper lobe cavitary lesions. His sputum
is positive for acid fast bacilli. A CBC shows: Hgb 14.2 g/dL, Hct 42.5%, MCV 92 fL,
platelet count 225,000/uL, and WBC count 44,500/uL with differential count of 59 segs,
20 bands, 8 metas, 4 myelos, 2 promyelos, 5 lymphs, and 2 monos. A bone marrow
biopsy is performed and on microscopic examination shows myeloid hyperplasia with
<5% blasts. Which of the following laboratory test findings is most likely to be present
in this man?


A     Elevated leukocyte alkaline phosphatase (A) CORRECT. The findings point to a
leukemoid reaction that can be seen with infections such as tuberculosis.


B     Karyotype with 46, XY, t(9;22)
C     Monoclonal gammopathy


D     Elevated D-dimer


E     Positive TdT

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Question 28

A 5-year-old boy has become increasingly lethargic for the past 2 months. On physical
examination his temperature is 37.3 C. There are ecchymoses noted on the skin of his
lower legs. Laboratory studies show Hgb 9.2 g/dL, Hct 27.8%, MCV 91 fL, platelet
count 101,000/microliter, and WBC count 12,128/microliter. A bone marrow biopsy is
performed and on microscopic examination shows nearly 100% cellularity with
replacement by primitive cells that have large nuclei with delicate chromatin and
indistinct nucleoli with scanty cytoplasm. These cells mark for CD10 (CALLA)
antigen. Which of the following is the most likely diagnosis?


A     Acute myeloid leukemia


B     Hodgkin lymphoma


C     Acute lymphoblastic leukemia (C) CORRECT. He has ALL, which typically
occurs in children. The CALLA variety is common and responds well to therapy.


D     Epstein-Barr virus infection


E     Leukemoid reaction


F     Chronic lymphocytic leukemia

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Question 29

A 79-year-old woman has developed increasing dyspnea for the past 2 weeks. On
physical examination she has diffuse rales in all lung fields. A CBC shows Hgb 7.1, Hct
22.2, MCV 93 fL, platelet count 205,000/microliter, and WBC count 6500/microliter
with differential count of 60 segs, 4 bands, 25 lymphs, 9 monos, and 2 eos with 10
nucleated RBCs/100 WBCs. Which of the following is the most likely diagnosis?


A     Iron deficiency anemia


B     Pernicious anemia


C     Anemia of chronic disease


D     Sickle cell anemia


E     Hemolytic anemia (E) CORRECT. She may have an autoimmune hemolytic
anemia, or a leukemia or lymphoma may be present. The marked anemia has led to
high output congestive heart failure with pulmonary edema.

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Question 30

A 40-year-old man has had constant dull pain in his lower right back for the past 4
months. On physical examination there is tenderness on percussion of his right
costovertebral angle. An abdominal CT scan reveals a 6 cm mass in the upper pole of
the right kidney. A CBC shows: Hgb 21.3 g/dL, Hct 64.0%, MCV 96 fL, platelet count
199,000/microliter, and WBC count 8230/microliter. Serum chemistries include sodium
141 mmol/L, potassium 4.3 mmol/L, chloride 102 mmol/L, CO2 25 mmol/L, glucose
77 mg/dL, urea nitrogen 18 mg/dL, and creatinine 1.3 mg/dL. Which of the following is
the most likely diagnosis?


A     Polycythemia vera


B     Erythroleukemia


C     Hemophilia A


D     Dehydration


E      Increased erythropoietin (E) CORRECT. He has a secondary form of
polycythemia. Renal cell carcinomas are known to secrete erythropoietin and lead to
this paraneoplastic effect with polycythemia.

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Question 31

A 38-year-old woman has become increasingly fatigued for the past 3 months. During
the past week she has noted purple blotches on her skin. On physical examination there
are purpuric areas of skin on her trunk and extremities. She has no hepatosplenomegaly
and no lymphadenopathy. Laboratory studies show Hgb 6.8 g/dL, Hct 20.7%, MCV 91
fL, platelet count 28,760/microliter, and WBC count 1940/microliter. Which of the
following is the most likely diagnosis?


A     Aplastic anemia (A) CORRECT. There is evidence from the peripheral
cytopenias for marked hypocellularity of the bone marrow with aplastic anemia. The
spleen is of normal size.


B     Chronic myelogenous leukemia


C     Infectious mononucleosis


D     Large B cell lymphoma


E     Hereditary spherocytosis

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Question 32

A 45-year-old man has noted a change in the appearance of his face over the past 7
months. On physical examination his facial skin is thickened and reddened. A punch
biopsy of skin is performed and on microscopic examination shows infiltration by
neoplastic T lymphocytes. Which of the following is the most likely diagnosis?


A     Hodgkin lymphoma


B     Mycosis fungoides (B) CORRECT. If these neoplastic cells circulate, it is known
as Sezary syndrome.


C     Burkitt lymphoma


D     Acute lymphocytic leukemia
E     Hairy cell leukemia

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Question 33

A 49-year-old man has had increasing fatigue for the past 4 months. On physical
examination he has massive splenomegaly but no lymphadenopathy. Laboratory studies
show a Hgb of 10.1 g/dL, Hct 30.3%, MCV 90 fL, WBC count 1600/microliter, and
platelet count 48,000/microliter. Examination of his peripheral blood smear shows
increased numbers of peripheral blood lymphocytes containing tartrate-resistant acid
phosphatase. Which of the following is the most likely diagnosis?


A     Chronic lymphocytic leukemia


B     HTLV-1 infection with leukemia


C     Hairy cell leukemia (C) CORRECT. The proliferation of B-lymphocytes involves
peripheral blood, bone marrow, and spleen. Splenomegaly is often present, but
hepatomegaly is not common, and lymphadenopathy is rare with hairy cell leukemia.


D     Gaucher disease


E     Myelodysplasia

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Question 34

A 10-year-old girl has exhibited increasing sluggishness with poorer performance in
school over the past year. She has not had increased numbers of infections. The child
now complains of headaches. A physical examination shows no hepatosplenomegaly or
lymphadenopathy. A CBC shows: Hgb 11.8 g/dL, Hct 33.9%, MCV 71 fL, platelet
count 293,000/microliter, and WBC count 8160/microliter. Examination of her
peripheral blood smear shows basophilic stippling of erythrocytes. The serum
haptoglobin is 5 mg/dL. Which of the following laboratory test findings is most likely
to be present in this girl


A     Hemoglobin S on electrophoresis
B     Increased osmotic fragility


C     Positive direct Coombs test


D      Decreased serum iron


E      Elevated free erythrocyte protoporphyrin (E) CORRECT. She has lead
poisoning. Lead inhibits incorporation of iron into heme, leading to elevated zinc
protoporphyrin and free erythrocyte protoporphyrin. The diminished heme synthesis
leads to a hypochromic, microcytic anemia, and there may be mild hemolysis.
Basophilic stippling can be an indicator of toxic injury to RBCs.

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Question 35

A 2-year-old boy has had a seborrheic eruption over the scalp and trunk over the past
month. He then develops a right ear ache. On physical examination the right tympanic
membrane is erythematous and bulging. He has hepatosplenomegaly and generalized
lymphadenopathy. Laboratory studies show Hgb 9.5 g/dL, Hct 28.7%, MCV 90 fL,
platelet count 58,000/microliter, and WBC count 3540/microliter. A bone marrow
biopsy is performed and on microscopic examination shows 100% cellularity with
extensive infiltration by cells resembling macrophages that express CD1a antigen and,
by electron microscopy, have prominent HX bodies (Birbeck granules). Which of the
following conditions is most likely to produce this boy's findings?


A      Myeloproliferative disorder


B     Plasmodium vivax infection


C     Hodgkin' disease, lymphocyte depletion type


D      Langerhans cell histiocytosis (D) CORRECT. The Langerhans cell histiocytoses
include Letterer-Siwe disease (as in this case, it is typically a disseminated disease of
children), and localized eosinophilic granuloma (often involving bone).


E     AIDS

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Question 36

A 72-year-old woman has had increasing fatigue with a 3 kg weight loss over the past 7
months. Her hands become purple and painful upon exposure to cold. On physical
examination she has a palpable spleen tip. Laboratory studies show Hgb 10.5 g/dL, Hct
31.7%, MCV 99 fL, platelet count 193,600/microliter, and WBC count 5390/microliter.
The direct Coombs test is positive at 4 C and negative at 37 C. Which of the following
underlying diseases is this woman most likely to have?


A      Non-Hodgkin lymphoma A) CORRECT. She has a cold autoimmune hemolytic
anemia. Though this may be idiopathic or due to an infection (Epstein-Barr virus;
Mycoplasma) a malignant lymphoma must be considered. Since the RBC's are being
sequestered in the spleen with extravascular hemolysis, there is splenomegaly. A warm
autoimmune hemolytic anemia will often produce intravascular hemolysis with
hemoglobinemia that will more often lead to renal failure. There will more often be
jaundice with intravascular hemolysis. Intravascular hemolysis will decrease
haptoglobin more.


B     Systemic lupus erythematosus


C     Pernicious anemia


D     Scleroderma


E     Thalassemia minor

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Question 37

A 12-year-old girl has the sudden onset of severe abdominal pain and back pain. On
physical examination her abdomen is diffusely tender, but there are no masses. She is
afebrile. A CBC shows Hgb 6.5 g/dL, Hct 19.0%, MCV 99 fL, platelet count
149,000/microliter, and WBC count 11,200/microliter. Examination of her peripheral
blood smear shows nucleated RBCs and sickled RBCs. Which of the following gene
mutations is she most likely to have?


A     Deletion


B     Duplication


C     Insertion
D     Missense (D) CORRECT. Missense mutations involve a change in a single
amino acid The defect in sickle cell anemia is a single amino acid substitution (valine for
glutamic acid), but the globin chain is still made. The gene may less commonly be
present in some Mediterranean and eastern Arabian populations. There is enough fetal
hemoglobin at birth to prevent sickling with sickle cell disease.


E      Nonsense


F     Splice site


G      Tandem repeat

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Question 38

A 32-year-old man has had worsening headaches for the past 2 months. On physical
examination he is afebrile. He has no lymphadenopathy or hepatosplenomegaly. A head
CT scan reveals a 3 cm mass lesion to the right of midline next to the lateral ventricle.
A stereotaxic brain biopsy is performed and microscopic examination shows diffuse
large B cell lymphoma. A bone marrow biopsy is performed and on microscopic
examination shows slightly decreased cellularity of all cell lines. Which of the
following laboratory test findings is this patient most likely to have?


A      Elevated terminal deoxyribonucleodidyl transferase


B      Bence-Jones proteinuria


C      Elevated serum IgM


D      HIV-1 RNA of 8000 copies/mL (D) CORRECT. He has a cerebral lymphoma
typical for AIDS with HIV infection.


E      Lymphoma positive for tartrate-resistant acid phosphatase

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Question 39

A 28-year-old African-American woman has had worsening fatigue for the past 2
months. On physical examination she has an erythematous macular rash on her upper
chest, forearms, and face. Laboratory studies show Hgb 9.2 g/dL, Hct 27.9%, MCV 101
fL, platelet count 179,000/microliter, and WBC count 5850/microliter. The red blood
cell distribution width is markedly increased. Her peripheral blood smear shows
polychromasia. Her reticulocyte count is 4.2%. The serum haptoglobin is 3 mg/dL.
Serum chemistries show total protein 7.9 g/dL, albumin 3.8 g/dL, alkaline phosphatase
49 U/L, AST 81 U/L, ALT 27 U/L, total bilirubin 3.3 mg/dL, and direct bilirubin 0.8
mg/dL. Hemoglobinuria is detected on urinalysis. Which of the following underlying
conditions is she most likely to have?


A     Multiple myeloma


B      Systemic lupus erythematosus (B) CORRECT. These findings all point to
intravascular hemolysis that can be seen with warm autoimmune hemolytic anemia that
can complicate autoimmune diseases such as SLE.


C     Hepatitis C infection


D     Hereditary spherocytosis


E     Vitamin B12 deficiency

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Question 40

A 37-year-old man known to be infected with HIV for the past 10 years has had
abdominal pain for the past 3 days. Physical examination reveals abdominal distension
with diffuse tenderness and absent bowel sounds. An abdominal CT scan reveals a mass
lesion involving the small intestine. He is taken to surgery, and an area of bowel
obstruction in the ileum is removed. Gross examination of the specimen shows a near-
encircling firm white mass 10 cm long and 3 cm in greatest depth that infiltrates
through the wall of the bowel. Which of the following neoplasms is this man most
likely to have?


A     Plasmacytoma


B     Hodgkin lymphoma, lymphocyte predominant type


C      High-grade B cell lymphoma (C) CORRECT. Such lymphomas are typical for, as
well as diagnostic of, AIDS in the setting of HIV infection. Extranodal involvement is
common
D      Metastatic adenocarcinoma


E     Myeloproliferative disorder


F     Adenocarcinoma

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Question 41

A 20-year-old healthy man incurs blunt force trauma to the abdomen in a motor vehicle
accident. On physical examination he has upper abdominal tenderness. An abdominal
CT scan reveals a splenic hematoma. At laparotomy a splenectomy is performed.
Following splenectomy, which of the following peripheral blood morphologic findings
is most likely to be present?


A      Tear drop cells


B     Elliptocytes


C     Target cells


D      Macro-ovalocytes


E     RBC inclusions (E) CORRECT. Howell-Jolly bodies and Pappenheimer bodies
are seen within RBC's in splenectomized patients. A functioning spleen would normally
remove such inclusions.

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Question 42

A 10-year-old girl is noted to have increasing facial distortion for the past 8 months
from a lesion involving her jaw. On physical examination she has a right mandibular
mass. A biopsy is performed and on microscopic examination reveals a monotonous
pattern of small non-cleaved lymphocytes. Infection with which of the following
organisms is most likely to be associated with development of this girl's mass lesion?


A      Adenovirus
B      Cytomegalovirus


C       Epstein-Barr virus (C) CORRECT. Burkitt lymphoma is endemic in Africa, mainly
seen in children and young adults, and EBV infection is implicated in the pathogenesis
of this B-cell lymphoma. Although Burkitt lymphoma is one of the fastest growing
neoplasms, half of cases do respond to chemotherapy with long-term survival.


D      Hepatitis C virus


E      Herpes simplex virus


F      HIV


G      HTLV-1

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Question 43

Two teenage siblings in the same family are noted to have frequent nosebleeds and easy
bruising from even minor trauma. The girl has had menorrhagia since menarche. The
girl's CBC shows Hgb 14 g/dL, Hct 42.3%, MCV 90 fL, platelet count
242,000/microliter, and WBC count 7720/microliter. Her prothrombin time is 12
seconds and partial thromboplastin time 25 seconds. Platelet function studies show
decreased aggregation in response to ADP, collagen, epinephrine, and thrombin. Which
of the following disorders are these siblings most likely to have?


A      Hemophilia A


B      Antithrombin III deficiency


C       Glanzmann thrombasthenia (C) CORRECT. Glanzmann thrombasthenia is an
autosomal recessive disorder from abnormalities in platelet glycoprotein IIb or IIIa,
which form a receptor that is required for platelet aggregation, for uptake of fibrinogen
into platelets, and clot retraction. This is a qualitative platelet defect; there are normal
numbers of circulating platelets.


D      Systemic lupus erythematosus


E      Von Willebrand disease
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Question 44

A 46-year-old man has been chronically fatigued for the past year. A physical
examination yields no abnormal findings. A CBC shows: Hgb 10.8 g/dL, Hct 33.1%,
MCV 104 fL, platelet count 239,000/microliter, and WBC count 7720/microliter. His
peripheral blood smear shows normal WBC morphology and RBCs with mild
poikilocytosis and a few target cells. His serum vitamin B12 is 512 pg/mL and folate
4.7 ng/mL. His serum haptoglobin is 151 mg/dL. Direct and indirect Coombs tests are
negative. Which of the following underlying conditions is most likely to explain his
findings?


A     Chronic lymphocytic leukemia


B     Peptic ulcer disease


C     Lead poisoning


D      Chronic alcoholism (D) CORRECT. There is mild macrocytosis with normal B12
and folate, without evidence for significant hemolysis. Liver disease can lead to RBC
membrane abnormalities, with the presence of a few target cells on the peripheral blood
smear. Chronic alcohol abusers may also have poor diets lacking in folate. An MCV
>100 fL is much more likely to result from megaloblastic anemia (B12 or folate
deficiency).


E     Hereditary spherocytosis

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Question 45

A 59-year-old man has had back pain for the past 6 months. On physical examination
his prostate is palpably enlarged and firm on digital rectal examination. Laboratory
studies show his serum prostate specific antigen is 23 ng/mL. His CBC shows Hgb 9.4
g/dL, Hct 28.1%, MCV 89 fL, platelet count 135,000/microliter, and WBC count of
8800/microliter with WBC differential count of 61 segs, 15 bands, 14 lymphs, 5 monos,
3 metas, and 2 myelos with 4 NRBCs/100 WBCs. Which of the following is the most
likely diagnosis?


A     Iron deficiency anemia
B      Leukoerythroblastosis (B) CORRECT. The immature WBC's and RBC's in the
peripheral blood are typical for leukoerythroblastosis. He probably has widely metastatic
prostate cancer to bone marrow.




C     Acute myelogenous leukemia


D      Disseminated intravascular coagulation


E     Leukemoid reaction

				
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