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A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 1 of 5
Which of the following is the most common malignancy of childhood?
/ A. Ewing sarcoma
/ B. Leukemia
/ C. Neuroblastoma
/ D. Retinoblastoma
/ E. Wilms tumor
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Explanation - Q: 1.1
Close
The correct answer is B. Leukemia is the most common malignancy of
childhood. The other malignancies listed are also characteristically childhood
diseases, but occur less frequently.
Ewing sarcoma (choice A) can involve bone or soft tissues;
Neuroblastoma (choice C) can involve the adrenal gland or the extraadrenal sympathetic chain.
Retinoblastoma (choice D) involves the eye, and Wilms tumor (choice E)
involves the kidney.
A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 2 of 5
The complete blood count shows anemia, thrombocytopenia, and a white count
of 110,000/mm3. The peripheral smear contains numerous
blasts, which are smalI, intensely blue cells with homogeneous chromatin,
regular nuclear shape, small or absent nucleolus, and scanty blue
cytoplasm. This patient's disease most likely will be classified as which of the
following types?
/ A. L1
/ B. L2
/ C. L3
/ D. M3
/ E. M7
Explanation - Q: 1.2
Close
The correct answer is A. These blasts cells are the lymphoid L1 cells, and
the child has ALL, type L1. L1 is one of the French-American-British
classifications for lymphoblastic leukemia, which also includes L2 and L3
types.
L2 (choice B) cells are large and heterogenous blast class with
heterogeneous chromatin, irregular nuclear shape, and large nucleolus.
L3 (choice C) cells are large and homogeneous cells whose most prominent
feature is cytoplasmic vacuolization that may overlie the nucleus.
A French-American-British classification has also been used for acute
myeloid leukemias, and different types are given different M designations.
M3 (choice D) is acute promyelocytic leukemia, and the cells have abundant
cytoplasm that often contains numerous Auer rods (small rod like structures).
M7 (choice E) is megakaryoblastic leukemia, and the cells are very large
and maybe multinucleated, resembling megakaryocytes.
A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 3 of 5
Which of the following is the most common form of leukemia in childhood?
/ A. Acute lymphoblastic leukemia
/ B. Acute myeloblastic leukemia
/ C. Chronic lymphocytic leukemia
/ D. Chronic myelogenous leukemia
/ E. Erythroleukemia
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Explanation - Q: 1.3
Close
The correct answer is A. Acute lymphoblastic leukemia is the most
common form of leukemia in children, and accounts for nearly 1/3 of all
pediatric cancers.
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A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 4 of 5
What is the peak age of incidence of the most common form of this disease in
children?
/ A. Less than 6 months
/ B. 8 -14 months
/ C. 2-5 years
/ D. 10-12 years
/ E. More than 15 years
Explanation - Q: 1.4
Close
The correct answer is C. While acute lymphoblastic leukemia can occur at
any age, its peak age of incidence is 2 to 5 years.
A child is taken to a pediatrician because the mother is concerned that the child
has been losing, rather than gaining weight, and has been
having numerous infections. After a complete physical examination with
screening serum chemistry and blood studies, the physician
suspects that the child has a malignancy.
Question 5 of 5
What is the cure rate of the most common form of this disease in children?
/ A. Less than 5%
/ B. 10-25%
/ C. 35-50%
/ D. 70-80%
/ E. More than 95%
Explanation - Q: 1.5
Close
The correct answer is D. With modern chemotherapeutic regimens, we
have now achieved a 70-80% cure rate of acute lymphoblastic leukemia. The
medical regimens with which we treat these children may include
corticosteroids (prednisone, dexamethasone), anti-neoplastic agents
(vincristine, asparaginase, daunorubicin, 6-mercaptopurine, cytarabine,
etoposide, cyclophosphamide), and prophylactic antimicrobials
(sulfamethoxazole and trimethoprim, nystatin, clotrimazole, itraconazole).
Radiotherapy is also sometimes used in these patients. Complications of
acute lymphoblastic leukemia or its treatment can include suppression of
normal bone marrow elements (predisposing for anemia, neutropenia, and
thrombocytopenia), tumor lysis syndrome (which produces hyperuricemia
and electrolyte abnormalities and can be prevented or ameliorated with
hydration and allopurinol), neurotoxicity (which may cause a mild mental
retardation and poor emotional control), and secondary malignancies.
A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 1 of 5
Which of the following is the most likely diagnosis?
/ A. Aplastic anemia
/ B. Folate deficiency anemia
/ C. Iron deficiency anemia
/ D. Sickle cell anemia
/ E. Thalassemia
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Explanation - Q: 2.1
Close
The correct answer is A. This patient most likely has aplastic anemia, which
is defined as an anemia that involves all cell lines produced in the marrow.
The underlying problem is an impaired ability of the most primitive stem cells
in the bone marrow (from which all the blood cell lines except lymphocytes
are derived) to reproduce. Patients may present with symptoms related to
anemia (pallor, headache, palpitations, dyspnea, or fatigue),
thrombocytopenia (mucosal bleeding or petechiae), or neutropenia
(infections, mouth and pharyngeal ulcerations). There is a double peak of
incidence, in the early 20's and then in older adulthood.
Folate deficiency (choice B) produces a megaloblastic anemia, and altered
neutrophils with hypersegmentation of the nucleus may be seen, but the
marrow will not be hypocellular.
Iron deficiency anemia (choice C) tends to be microcytic, and does not affect
cell lines other than erythrocytes.
Sickle cell anemia (choice D) has bizarre-shaped erythrocytes and affects
only the erythrocyte line.
Thalassemia (choice E) is characterized by hypochromic erythrocytes that
may have a "target" appearance, and does not affect cell lines other than the
erythrocytic line.
A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 2 of 5
Which of the following is noted for sometimes causing this patient's condition?
/ A. Asbestos
/ B. Benzene
/ C. Lead
/ D. Mercury
/ E. Methyl alcohol
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Explanation - Q: 2.2
Close
The correct answer is B. Approximately 80% of cases of aplastic anemia
are acquired, with the remainder being congenital. Among the acquired
cases, often no inciting cause can be identified, and an autoimmune basis
appears probable. However, in some cases, an inciting cause can be
identified, and in these, it is important to recognize and if possible remove
the cause. Benzene is the chemical most commonly cited as being
associated with development of aplastic anemia.
Associate asbestos (choice A) with pulmonary fibrosis, mesothelioma, and
lung cancer.
Associate lead poisoning (choice C) with a hypochromic, microcytic anemia,
abdominal discomfort, and CNS effects.
Associate mercury poisoning (choice D) with (depending upon the route of
exposure) GI disturbance, pneumonitis, renal failure, and CNS involvement.
Associate methyl alcohol (choice E) with visual system and central nervous
system damage.
A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 3 of 5
Which of the following medications has been associated with the development of
this patient's condition?
/ A. Amyl nitrate
/ B. Aspirin
/ C. Chloramphenicol
/ D. CIofazimine
/ E. Dapsone
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Explanation - Q: 2.3
Close
The correct answer is C. Drugs noted for causing aplastic anemia include
chloramphenicol, phenylbutazone, and gold. Cases triggered by idiosyncratic
reaction to drugs appear not to involve an immune mechanism, but may be a
result of direct toxicity, possibly involving altered detoxification.
Amyl nitrate (choice A) does not have any hematologic side effects.
Aspirin (choice B) can cause leukopenia, thrombocytopenia, and
prolongation of bleeding time, but not aplastic anemia.
Clofazimine (choice D) does not have any hematologic side effects.
Dapsone (choice E) can cause dose-related hemolysis in patients with or
without glucose-6-phosphatase deficiency.
A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 4 of 5
With supportive care alone, this patient's condition is estimated to have which of
the following mortality rates?
/ A. Less than 5%
/ B. 10-25%
/ C. 30-40%
/ D. 50-60%
/ E. More than 70%
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Explanation - Q: 2.4
Close
The correct answer is E. Supportive care consists primarily of appropriate
transfusions to replace life-threateningly low red cell counts or platelet
counts. Supportive care alone is no longer considered adequate therapy,
since aplastic anemia has a greater than 70% mortality rate if that is the only
therapy offered.
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A 22-year-old man has been having fatigue and headaches. On physical
examination, the patient is noted to be pale and has a petechial
rash. A complete blood count and peripheral smear show markedly decreased
numbers of platelets, red blood cells, granulocytes,
monocytes, and reticulocytes. The erythrocyte morphology is normaI. Bone
marrow aspiration and biopsy show a marrow with 10% cellularity,
the rest of the marrow being occupied principally by fat cells.
Question 5 of 5
If treated with immunosuppression, the five year survival rate is approximately
which of the following?
/ A. 5%
/ B. 25%
/ C. 50%
/ D. 75%
/ E. 95%
Explanation - Q: 2.5
Close
The correct answer is D. Patients treated with immunosuppression have an
estimated 75% 5-year-survival rate, and those lucky individuals who have
matched sibling donor bone marrow transplant have a more than 90% 5year-survival. Colony stimulating cytokines (G-CSF and GM-CSF) are now
commercially available, and can also have great value in stimulating any
residual growth capacity the stem cells have.
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A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 1 of 8
Which of the following is the most likely diagnosis?
/ A. Acute lymphocytic leukemia
/ B. Acute myelogenous leukemia
/ C. Chronic lymphocytic leukemia
/ D. Chronic myelogenous leukemia
/ E. Hodgkin disease
Explanation - Q: 3.1
Close
The correct answer is D. This patient most likely has chronic myelogenous
leukemia (CML). This is a cancer of pluripotent marrow stem cells
characterized by a proliferation, predominantly of granulocytes, although
other cell lines (megakaryocytes, monocytes, erythrocytes, and even some T
and B cell lines) may also be increased.
Blast cells would be seen in the peripheral smear in acute myelogenous
leukemia (choice B) and acute lymphocytic leukemia (choice A).
In chronic lymphocytic leukemia (choice C), the numbers of lymphocytes are
increased and the numbers of other blood cells may be decreased.
Hodgkin disease (choice E) may show a modest increase in neutrophils and
eosinophils in the blood, but would not have the dramatic increase seen in
this patient.
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A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 2 of 8
Which of the following is the median age for presentation of this disease?
/ A. 1 year
/ B. 5 years
/ C. 25 years
/ D. 45 years
/ E. 75 years
Explanation - Q: 3.2
Close
The correct answer is D. Chronic myelogenous leukemia is primarily a
disease of middle age, with a median age of 45 years. The comparatively
few cases occurring in the elderly are more likely to be aggressive than those
occurring in middle-aged adults.
A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 3 of 8
This patient's disease can be confused in some settings with polycythemia vera.
Which of the following tests would be most helpful in
distinguishing between these two diseases?
/ A. Leukocyte alkaline phosphatase
/ B. Myelin basic protein
/ C. Protein C activity
/ D. ScI-70
/ E. Tartrate resistant acid phosphatase
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Explanation - Q: 3.3
Close
The correct answer is A. While to the novice, polycythemia vera and
chronic myelogenous leukemia appear to be obviously different diseases,
both cause an increase in many marrow cell lines, and ambiguous cases do
exist. Leukocyte alkaline phosphatase is a helpful test to perform in these
settings, because it is characteristically low in chronic myelogenous
leukemia, but high in polycythemia vera. Also, polycythemia vera does not
usually have the Philadelphia chromosome.
Myelin basic protein (choice B) is used in the diagnosis of multiple sclerosis.
Protein C (choice C) is involved in the clotting mechanism.
Scl-70 (choice D) is an antibody often present in scleroderma.
Associate tartrate-resistant acid phosphatase (choice E) positivity with hairy
cell leukemia.
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A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 4 of 8
Which of the following organs is most commonly significantly enlarged in these
patients?
/ A. Liver
/ B. Pancreas
/ C. Stomach
/ D. Spleen
/ E. Thymus
Explanation - Q: 3.4
Close
The correct answer is D. Splenomegaly is common in patients with chronic
myelogenous leukemia, and in more than half of the patients, is palpable
more than 5 cm below the costal margin at the time of diagnosis.
Splenomegaly may be a very helpful sign on physical examination, since it is
uncommon in reactive leukocytosis. Hepatomegaly (choice A) can also
occur, but is less common. The other answers are distracters.
A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 5 of 8
Which of the following translocations is very common in this patient's disease?
/ A. t(8;14)
/ B. t(8;21)
/ C. t(9;22)
/ D. t(12;21)
/ E. t(15;17)
Explanation - Q: 3.5
Close
The correct answer is C. The characteristic chromosomal translocation of
chronic myelogenous leukemia is t(9;22)(q34;q11). This translocation results
in a distinctive abnormal chromosome sometimes called the Philadelphia
chromosome, and is seen in 90-95% of CML cases. The Philadelphia
translocation is also seen in 20-25% of adults with acute lymphoblastic
leukemia, 2% of adults with acute myeloid leukemia, and 2-10% of childhood
acute lymphoblastic leukemia. Some authors think that these cases may
represent variants of chronic myelogenous leukemia in which the "blast
transformation" (which commonly occurs 3-5 years after diagnosis in
untreated patients with chronic myelogenous leukemia) either develops very
early in the disease, or later, but in a patient in whom the chronic phase had
been missed clinically. The Philadelphia translocation is not usually
encountered in children with acute myeloid leukemia or in adults with chronic
lymphocytic leukemia.
T(8;14) (choice A) is a translocation with unfavorable prognosis found in
some cases of acute lymphoblastic (L3 form) leukemia.
T(8;21) (choice B) is a translocation with favorable prognosis found in some
cases of acute myelogenous leukemia (M2 form).
T(12;21) (choice D) is a translocation with favorable prognosis found in
some cases of acute lymphoblastic (L1 form) leukemia.
T(15;17) (choice E) is a translocation with favorable prognosis found in
some cases of acute myelogenous leukemia (M3 form).
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A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 6 of 8
This translocation joins which of the following two genes?
/ A. BCR and ABL
/ B. E2A and PBX1
/ C. myc and lgH
/ D. myc and kappa light chain
/ E. myc and lambda light chain
Explanation - Q: 3.6
Close
The correct answer is A. The t(9;22) Philadelphia translocation links the
Abelson oncogene (c-ABL) (normally on chromosome 9) to the BCR region
of chromosome 22. The resulting gene product is a new, chimeric, protein
called BCR-ABL, which has tyrosine kinase activity. In some manner still not
well understood, this gene product then produces the clinical problems we
diagnosis as CML.
E2A and PBX1 (choice B) describes t(1;19), seen in some cases of pre-Bcell ALL, which links the E2A gene product (a basic helix-loop-helix
transcription factor) with the homeobox gene PBX1.
The myc oncogene and the immunoglobulin heavy chain IgH (choice C) are
joined in a t(8;14) translocation commonly seen in Burkitt's lymphoma and
type L3 acute lymphoblastic leukemia.
The translocations described in choices D and E also occur with lower
frequency in Burkitt's lymphoma and L3 acute lymphoblastic leukemia; in
these cases, the translocations produced are, respectively, t(2;8) and t(8;22).
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A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 7 of 8
Which of the following specifically inhibits the abnormal gene product produced
by the translocation seen in this disease?
/
/
/
/
/
A. Busulfan
B. Hydroxyurea
C. Imatinib mesylate
D. Interferon alfa-2a
E. Interferon alfa-2b
Explanation - Q: 3.7
Close
The correct answer is C. Imatinib mesylate (Gleevec) is an important new
drug that specifically targets the abnormal gene product (a tyrosine kinase)
of the BCR-ABL translocation. This new drug is well absorbed after oral
administration and can be used to treat both the chronic phase and blast
crisis of CML. It also shows promise in potentially being used in autologous
(self-to-self) bone marrow transplant, where it may be possible to clear the
transplant of leukemic cells in vitro with chemotherapy before reinjecting the
transplant. The other agents listed in the choices are also used in the
chemotherapy of CML.
Busulfan (choice A) is an alkylating agent, hydroxyurea (choice B) inhibits
DNA synthesis, and recombinant forms of interferons (choices D and E) can
help induce remissions in CML.
A complete blood count (CBC) and a blood smear are taken from a patient who
has been complaining of fatigue. The CBC shows a white
count of 200,000/mm3, with differential count including 70% segmented
neutrophils and 15% band forms. The absolute numbers of basophils
and eosinophils are increased somewhat. Review of the peripheral smear does
not show any blast forms.
Question 8 of 8
The cure rate with patients who have this condition and undergo bone marrow
transplant is now estimated to be which of the following?
/ A. 5%
/ B. 15%
/ C. 25%
/ D. 50%
/ E. 80%
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Explanation - Q: 3.8
Close
The correct answer is D. In the past, the average life expectancy of CML
patients has been 3-5 years, with most patients dying after a blast crisis
develops. The blast crisis may produce either clinical acute lymphoblastic
leukemia or acute myeloid leukemia. This was historically true despite
chemotherapy and any apparent improvements in blood counts with
chemotherapy. Bone marrow transplant is becoming an increasingly
preferred treatment modality in those cases in which a suitable donor can be
found, because it now has a cure rate of approximately 50%. The mortality
rate of this procedure is 10-20% or less if a matched sibling donor is used
and increases to 30-40% if an unrelated donor is used.
A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 1 of 5
Which of the following enzymatic deficiencies would most likely cause a
hemolytic episode such as this man is experiencing?
/ A. Acid sphingomyelinase deficiency
/ B. GIucocerebrosidase deficiency
/ C. GIucose-6-phosphate dehydrogenase deficiency
/ D. Ferrochelatase deficiency
/ E. Phytanic acid alpha-oxidase deficiency
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Explanation - Q: 4.1
Close
The correct answer is C. Of the enzyme deficiencies listed, only glucose-6phosphate dehydrogenase deficiency (G-6-PD) would cause hemolysis. The
cells most likely to be affected are usually the older erythrocytes. Most
patients with this condition have long asymptomatic intervals and only
undergo hemolysis when a metabolic stress occurs. While elementary
discussions of this condition usually stress the ingestion of fava beans or the
taking of medicines (e.g., sulfonamides, antimalarial drugs) as triggers of
hemolysis, you should be aware that in real life in the United States, the most
common triggers are fever, acute viral and bacterial infections, and diabetic
ketoacidosis. Once the diagnosis of G6PD deficiency is suspected, the
actual enzyme activity can be assayed in the laboratory to establish the
diagnosis.
Acid sphingomyelinase deficiency (choice A) produces the lysosomal
storage disease Niemann-Pick disease.
Glucocerebrosidase deficiency (choice B) produces the lipid storage
disease Gaucher disease.
Ferrochelatase deficiency (choice D) produces erythropoietic protoporphyria.
Phytanic acid alpha-oxidase deficiency (choice E) produces the storage
disease Refsum disease.
A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 2 of 5
Which of the following is the approximate incidence of this enzymatic defect in
African-American males?
/ A. Less than 0.1%
/ B. 1%
/ C. 10%
/ D. 30%
/ E. 90%
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Explanation - Q: 4.2
Close
The correct answer is C. The incidence of glucose-6-phosphate deficiency
in American black males is thought to be about 12%. The condition can also
be found in lower frequency in black females (who either have both X
chromosomes involved or who have one affected X chromosome with the
other inactivated as a Barr body in a high percentage of the body). G6PD
deficiency has a worldwide distribution, but occurs most frequently in tropical
and subtropical areas of the Eastern hemisphere. Populations that have a
significant frequency of G6PD deficiency include Brazilian Blacks, people
from the lowlands of the Mediterranean basin, especially those of Greek or
Sardinian descent, Kurdish Jews and the Southern Chinese. Most affected
individuals are asymptomatic. However, individuals from ethnic backgrounds
with high frequencies of G6PD deficiency should be checked for it if
treatment of an illness requires a medication known to precipitate hemolysis.
G6PD deficiency should be also be considered in the differential diagnosis of
any nonimmune hemolytic anemia.
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A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 3 of 5
Which of the following is a product of the enzyme for which the patient has a
deficiency?
/ A. FADH2
/ B. NAD+
/ C. NADH
/ D. NADP+
/ E. NADPH
Explanation - Q: 4.3
Close
The correct answer is E. Glucose-6-phosphate dehydrogenase is the
committed step of the hexose monophosphate shunt pathway (pentose
phosphate pathway). The enzymes of this pathway are present in the
cytoplasm of all cells, but are especially prevalent in adipose and liver cells.
The hexose monophosphate shunt pathway makes ribose-5-phosphate for
nucleotide synthesis, interconverts 6 carbon sugars and 5 carbon sugars,
and produces NADPH. NADPH, the reduced form of nicotinamide adenine
dinucleotide phosphate, is used in reductive biosynthetic reactions such as
fatty acid synthesis, and in the regeneration of glutathione by glutathione
reductase. Glutathione has three main roles in a cell: it keeps the protein
sulfhydryl groups of cysteines reduced, it reduces peroxides, and it maintains
hemoglobin in the reduced state.
NADP+ (choice D) is also involved in this reaction, but as a substrate, rather
than a product.
The various forms of NAD (choices B and C) and FAD (choice A) do not
take part.
A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 4 of 5
Regeneration of which of the following in a reduced form is impaired in
individuals with this disorder?
/ A. Acetaldehyde
/ B. AcetyI CoA
/ C. Biotin
/ D. GIutathione
/ E. GIycogen
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Explanation - Q: 4.4
Close
The correct answer is D. Reduced glutathione is an effective scavenger of
dangerous oxidative metabolites; regeneration of reduced glutathione is
accomplished by glutathione reductase with the aid of NADPH, a product of
the hexose monophosphate shunt pathway. Red blood cells that are deficient
for G6PD, and are exposed to oxidants, can be depleted of their store of
glutathione. The lack of glutathione leads to the oxidation of the sulfhydryl
groups on hemoglobin, causing the formation of methemoglobin and then
denatured globin, which forms insoluble masses called Heinz bodies that are
attached to the RBC membrane. The overall outcome is that the red blood
cells become rigid, making them susceptible to destruction by the
reticuloendothelial system.
The other answers are distracters.
A 17-year-old African American with type 1 diabetes spends a weekend camping
with friends. A day after returning, he is brought into the
emergency department because he is in diabetic ketoacidosis. In the emergency
room, he is noted to be jaundiced. The laboratory calls the
emergency department physician because the plasma has a red color.
Question 5 of 5
In a Nigerian population, the frequency of males with G6PD deficiency is 0.2.
Assuming the population is in Hardy-Weinberg equilibrium at
this locus, what is the frequency of homozygous females?
/ A. 0.02
/ B. 0.04
/ C. 0.2
/ D. 0.32
/ E. 0.45
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Explanation - Q: 4.5
Close
The correct answer is B. Glucose 6-phosphate dehydrogenase deficiency
is an X-linked recessive trait. A male is hemizygous for the X chromosome,
and thus has only one copy of each trait located on that chromosome. The
frequency of an X-linked recessive in males is thus equal to the frequency of
the allele in the population. From this, we know that q= 0.2 and p = 0.8. A
woman has two copies of each gene on the X chromosome, so the equation
for Hardy Weinberg equilibrium is the same as for autosomal traits. In this
case, a homozygous recessive female would occur at a frequency of q2 or
0.04.
Choice A, 0.02, is incorrect. If a person remembered that G6PD was more
frequent in males, but didn't know how to use the equations to get the true
estimate, he or she might guess this answer.
Choice C, 0.2, makes the assumption that the trait is autosomal, and so the
frequency of affected males and affected females are equal.
Choice D, 0.32, assumes that q=0.01, and then calculates the frequency of
carrier females (2pq).
Choice E, 0.45 is a distracter
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A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 1 of 5
Deficiency of which of the following would most likely cause this child's anemia?
/ A. Iron
/ B. Folate
/ C. Vitamin A
/ D. Vitamin C
/ E. Vitamin K
Explanation - Q: 5.1
Close
The correct answer is B. The combination of enlarged, hypochromic
erythrocytes with hypersegmented neutrophils indicates that the patient has
a megaloblastic anemia. Of the causes listed in the choices, only folate
deficiency will produce these changes. Folate deficiency can also be caused
by inadequate intake (elderly, alcoholics, infants), increased requirements
(pregnancy, infancy, high metabolic states), impaired utilization
(methotrexate therapy), or increased loss (hemodialysis).
Iron deficiency (choice A) usually produces a microcytic, hypochromic
anemia.
Vitamin A deficiency (choice C) causes night blindness.
Vitamin C deficiency (choice D) causes scurvy and poor wound healing.
Vitamin K deficiency (choice E) causes a bleeding tendency.
A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 2 of 5
Deficiency of which the following can also cause this patient's type of anemia?
/ A. Calcium
/ B. Magnesium
/ C. Vitamin B12
/ D. Vitamin D
/ E. Vitamin E
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Explanation - Q: 5.2
Close
The correct answer is C. Vitamin B12 deficiency can also cause
megaloblastic anemia.
Calcium and vitamin D deficiencies (choices A and D) can cause
osteoporosis and rickets.
Magnesium deficiency (choice B) is usually seen in settings in which
patients are very ill for other reasons, but appears to cause GI symptoms,
personality change, and tetany.
Vitamin E (choice E) is an antioxidant. Deficiency may produce mild
hemolytic anemia or neurologic (spinocerebellar) disease.
A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 3 of 5
The substance most likely deficient in this child is normally absorbed primarily at
which of the following sites?
/ A. Colon
/ B. Distal ileum
/ C. Duodenum and jejunum
/ D. Esophagus
/ E. Stomach
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Explanation - Q: 5.3
Close
The correct answer is C. Folate is absorbed in the proximal small bowel
and vitamin B12 is absorbed in the ileum (choice B). Thus poorly controlled
celiac disease, which principally affects the proximal small intestine, is more
likely to cause folate deficiency than B12 deficiency.
The esophagus (choice D) and stomach (choice E) are not major absorptive
sites; and the colon (choice A) principally absorbs fluid and electrolytes.
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A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 4 of 5
The two substances that can cause this type of anemia if deficient, are necessary
for synthesis of which of the following?
/ A. AcetyI CoA
/ B. ADP
/ C. ATP
/ D. NADPH
/ E. Tetrahydrofolate (THF)
Explanation - Q: 5.4
Close
The correct answer is E. Vitamin B12 is a cofactor in the activation of folic
acid to tetrahydrofolate (THF). The THF is necessary for DNA base
synthesis, and careful evaluation will demonstrate megaloblastic changes in
all 3 hematopoietic lines. The other answers are distracters.
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A follow-up physical examination is performed on a 7-year-old child with known
celiac disease, which has been difficult to controI. A complete
blood count is done as part of the evaluation, which demonstrates a moderate
anemia. Review of the peripheral blood smear shows
numerous enlarged, hypochromic erythrocytes. Some of the neutrophils show
hypersegmentation of the nuclei, with up to 8 Iobes, rather than
the usuaI 3.
Question 5 of 5
Deficiency of vitamin B12 can produce damage to which of the following?
/ A. Cardiovascular system
/ B. Central nervous system
/ C. Female reproductive system
/ D. Hepatobiliary system
/ E. Urinary system
Explanation - Q: 5.5
Close
The correct answer is B. Vitamin B12 functions in transfer of 1-carbon
fragments, and has a role in other pathways besides DNA synthesis. Vitamin
B12 deficiency can also cause demyelination and myelopathy, with resultant
CNS degeneration. The other choices are distracters.
_.
A 2-year-old African American child is evaluated by a pediatrician because the
mother noticed that the child had been behaving listlessly for
several months. Office hematocrit is 29%. A peripheral blood smear is shown
above.
Question 1 of 6
Which of the following is the most likely diagnosis?
/ A. AIpha thalassemia major
/ B. Beta thalassemia major
/ C. Beta thalassemia minor
/ D. Sickle cell anemia
/ E. Sickle cell trait
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Explanation - Q: 6.1
Close
The correct answer is D. This child has sickle cell anemia. This is an
autosomal recessive, chronic hemolytic anemia characterized by sickle- or
crescent-shaped erythrocytes. Almost all cases are individuals with at least
some African ancestry. In African Americans, the prevalence of sickle cell
anemia is about 0.3%.
The various thalassemias (choices A, B, and C) can be excluded because
the peripheral smear findings would include a reference to or a description of
target cells. In questionable cases, or when the possibility of coexisting
disease is present, hemoglobin electrophoresis can distinguish between the
various diseases noted in the choices.
Sickle cell trait (choice E), or the heterozygote and milder form of sickle cell
disease, can be excluded because the patient is frankly anemic with a very
low hematocrit. Sickle cell trait has a prevalence in African Americans of
about 8-13%. These individuals are usually asymptomatic, but may develop
clinical disease in unusual situations (such as with severe infections).
Question 2 of 6
The disease is caused by a nucleotide substitution at position +70 in the gene
region. The mutation also destroys a restriction endonuclease
site from +66 to +72 in the gene. Using a probe that binds just upstream from the
mutation, restriction patterns from his mother and from his
father were obtained and are shown at the left on the diagram below.
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Which of the patterns on the right most likely represents that of the child?
/ A. A
/ B. B
/ C. C
/ D. D
/ E. E
Explanation - Q: 6.2
Close
The correct answer is E. The mutation that causes the disease also
destroys a restriction enzyme site. Loss of a restriction site would produce a
longer restriction fragment that would be retained higher on the gel. Thus, in
both parents' patterns, the upper band must be cut from the chromosome
bearing the mutation. The affected child would be homozygous for this
mutation and show only the upper band in his pattern. The band is thicker,
indicating more material. The same size restriction fragment is cut from both
his chromosomes.
Choice A shows a homozygous normal individual.
Choice B shows an individual with a band representing a shorter fragment
not seen in either parent.
Choice C shows an individual with a band representing a longer fragment
not seen in either parent. If the mutation involved a triplet repeat expansion,
a pattern like this might be generated. It is not correct for the mutation
described in the stem.
Choice D shows a carrier.
Question 3 of 6
The prevalence of this disease in African Americans is about 1/625. The boy's
uncle has been tested and knows that he is a carrier of the
mutation involved. If he marries an African American woman, what is the risk
their first child will be affected with this disease?
/ A. 1/2500
/ B. 1/1200
/ C. 1/125
/ D. 1/100
/ E. 1/50
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Explanation - Q: 6.3
Close
The correct answer is E. If the prevalence of the disease (q2) were 1/625,
then the carrier frequency (2pq) in the same population would be 2(1/25) or
2/25
The risk of having an affected child is then: 2/25 x 1/4 = 2/100 or 1/50. The
risk is the same for each child, regardless of birth order.
Question 4 of 6
Compared to the prevalence in African Americans (1/625), the prevalence of this
disease in Africans may be as high as 1/50. Which of the
following genetic mechanisms contributes to this disparity?
/ A. Founder effect
/ B. Gene flow
/ C. Genetic drift
/ D. Incomplete penetrance
/ E. Increased mutation rate in Africans as compared
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Explanation - Q: 6.3b
to African Americans.
Close
The correct answer is E. If the prevalence of the disease (q2) were 1/625,
then the carrier frequency (2pq) in the same population would be 2(1/25) or
2/25
The risk of having an affected child is then: 2/25 x 1/4 = 2/100 or 1/50. The
risk is the same for each child, regardless of birth order.
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Question 4 of 6
Compared to the prevalence in African Americans (1/625), the prevalence of this
disease in Africans may be as high as 1/50. Which of the
following genetic mechanisms contributes to this disparity?
/ A. Founder effect
/ B. Gene flow
/ C. Genetic drift
/ D. Incomplete penetrance
/ E. Increased mutation rate in Africans as compared to African Americans.
Explanation - Q: 6.4
Close
The correct answer is B. Gene flow refers to the exchange of genes among
populations. When two populations, previously isolated reproductively, begin
to intermarry, gene frequencies within both populations will change due to
this flow, eventually reaching new equilibria. In either of the populations,
some gene frequencies will increase and some will decrease. Another cause
of the higher prevalence of sickle cell disease in Africans is the continued
selective pressure of malaria, not present in the U.S. Natural selection is not
given as an option in this question.
A founder effect (choice A) refers to the introduction of a mutation into a
rapidly expanding population by an individual who was part of a small
ancestral group. Both populations in this question share the same genetic
origin (Africa) and carry the same mutation. A founder effect would not
account for this disparity in gene frequency.
Genetic drift (choice C) refers to the random fluctuations of gene
frequencies in small populations. Some genes can increase in frequency,
others may decrease, when compared to the gene frequencies seen in larger
populations. Gene frequencies are statistical measurements, and small
populations are more likely to vary from the statistical mean than large
populations. Consider a family in which both parents are carriers of an
autosomal recessive disease-producing gene. Statistics indicate that if the
family has enough children, 25% will be AA, 50% will be Aa, and 25% will be
aa. If the family only has 4 children, there is some random chance that 2 will
be affected (e.g. 50% rather than 25%)
Incomplete penetrance (choice D) refers to the fact that not all individuals
with the disease-producing genotype have the disease (phenotype). The
same mutation should show nearly identical penetrance in two populations,
and would not account for the differences described in this question.
Mutation rates in large populations tend to be the same (compare to choice
E), and do not account for major differences in gene frequencies.
Question 5 of 6
Which of the following viruses most commonly causes aplastic crises in patients
with this disease?
/ A. BK virus
/ B. Human herpes virus 8
/ C. Human papilloma virus 18
/ D. JC virus
/ E. Parvovirus B19
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Explanation - Q: 6.5
Close
The correct answer is E. Sickle cell patients are vulnerable to a variety of
potentially life-threatening clinical crises. Important causes of aplastic crises
(also called hematologic crises), in which the marrow fails to produce
adequate numbers of erythrocytes, include parvovirus B19 infection and
folate deficiency. For your personal information, although the question is
usually asked about sickle cell anemia, these two inciting agents can also
induce exacerbation of anemia in individuals with thalassemia and a variety
of other hereditary red cell disorders. Simple transfusions can be used to
treat aplastic crises.
BK virus (choice A) causes renal disease in immunocompromised hosts.
Human herpes virus 8 (choice B) has been implicated in Kaposi sarcoma.
Human papilloma virus 18 (choice C) is an important cause of cervical
dysplasia and cancer.
JC virus (choice D) causes progressive multifocal leukoencephalopathy.
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Question 6 of 6
At age 5, the boy and his mother take a trip in a friend's small plane. On landing,
the boy complains of very severe pain involving his bones
and abdomen. Which of the following problems most likely developed?
/ A. Aplastic crisis
/ B. Hematologic crisis
/ C. Infectious crisis
/ D. Osteomyelitis
/ E. Vasoocclusive crisis
Explanation - Q: 6.6
Close
The correct answer is E. This patient probably developed a vasoocclusive
crisis, which occurs when there is an exacerbation of red cell sickling. The
increased sickling leads to obstruction of the microcirculation with sickled
erythrocytes, which in turn produces ischemia with its accompanying severe
pain (analogous to the pain of angina and myocardial infarction). The pain
most frequently involves bones, abdomen, and/or chest. Involvement of the
nervous system can produce focal neurologic signs and symptoms.
Involvement of the chest can produce acute chest syndrome (due to
microvascular occlusion in the pulmonary capillary bed), and is the major
cause of death in children less than 5 years of age who have sickle cell
disease. Precipitating causes for vasooclusive crises include cold weather
(which induces vasospasm), hypoxia (as in this patient who flew in an
unpressurized airplane), infection, dehydration, acidosis, alcohol intoxication,
emotional stress, and pregnancy. Vasoocclusive crises are usually treated
with hydration and analgesia. In severe cases, or if acute chest syndrome
develops, exchange blood transfusion is sometimes used to reduce the
number of circulating sickled cells. Important triggers for aplastic or
hematologic crises (choices A and B) are discussed in the explanation to
the preceding question. Infections (choice C), which can include
osteomyelitis (choice D), are usually related to these patients' functional
asplenia (due to splenic ischemia and infarction) and resultant defective
immunity. Encapsulated organisms (Haemophilus influenzae and
Streptococcus pneumoniae) are a particular vulnerability, but there is also an
increased vulnerability (apparently related to altered serum IgM levels,
impaired opsonization, and poor complement pathway function) to some
other organisms including Mycoplasma pneumoniae, Salmonella
typhimurium, Staphylococcus aureus, and Escherichia coli.
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A 62-year-old woman consults her physician because she has been having
headaches, dizziness, and tinnitus. The patient also reports that
her skin seems to be a great deal more itchy than several years ago, and that the
episodes of itchiness are sometimes triggered by a hot
shower. Physical examination demonstrates hypertension, splenomegaly, and a
ruddy complexion seen best in the face, palms, and nail
beds. The patient did not appear to be dehydrated.
Question 1 of 4
The patient has an erythrocyte count of 6.7 million/mm3. Which of the following
studies would be helpful in establishing that an absolute
increase in erythrocyte mass has occurred?
/ A. Free erythrocyte protoporphyrin
/ B. Hematocrit
/ C. Hemoglobin
/ D. Partial thromboplastin time
/ E. Radioactive chromium labeling
Explanation - Q: 7.1
Close
The correct answer is E. The dilution of small numbers of 51Cr-labelled
erythrocytes in the patient's blood can be used to calculate the patient's red
cell mass. This is important in cases of erythrocytosis, because it allows the
exclusion of hemoconcentration effects (relative erythrocytosis) not related to
a true increase in red cell mass.
Free erythrocyte protoporphyrin (choice A) is used in the evaluation of the
porphyrias.
Hematocrit (choice B) and hemoglobin (choice C) cannot distinguish
between a relative and an absolute erythrocytosis.
Partial thromboplastin time (choice D) is used in the evaluation of clotting
disorders.
A 62-year-old woman consults her physician because she has been having
headaches, dizziness, and tinnitus. The patient also reports that
her skin seems to be a great deal more itchy than several years ago, and that the
episodes of itchiness are sometimes triggered by a hot
shower. Physical examination demonstrates hypertension, splenomegaly, and a
ruddy complexion seen best in the face, palms, and nail
beds. The patient did not appear to be dehydrated.
Question 2 of 4
If the erythrocytosis is related to ectopic production of erythropoietin, which of the
following tumors would be the most likely source of the
erythropoietin?
/ A. Adenocarcinoma of the breast
/ B. Endometrial adenocarcinoma
/ C. Hepatocellular carcinoma
/ D. Renal cell carcinoma
/ E. Serous cystadenoma of the ovary
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Explanation - Q: 7.2
Close
The correct answer is D. Erythropoietin is normally produced in the kidneys,
and renal cell carcinoma can produce it ectopically, producing a form of
secondary erythrocytosis. The other answers are distracters.
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A 62-year-old woman consults her physician because she has been having
headaches, dizziness, and tinnitus. The patient also reports that
her skin seems to be a great deal more itchy than several years ago, and that the
episodes of itchiness are sometimes triggered by a hot
shower. Physical examination demonstrates hypertension, splenomegaly, and a
ruddy complexion seen best in the face, palms, and nail
beds. The patient did not appear to be dehydrated.
Question 3 of 4
In addition to the increased red cell count, this patient has a leukocytosis of 15 x
103per microliter, with increases in neutrophils, eosinophils,
and basophils. Her platelet count is 450 x 103per microliter. Which of the
following is most likely to be contributing to this woman's pruritus?
/ A. Basophils
/ B. Eosinophils
/ C. Erythrocytes
/ D. Neutrophils
/ E. PIatelets
Explanation - Q: 7.3
Close
The correct answer is A. This patient's disease is closely related to chronic
myelogenous leukemia, and increases in a variety of cell lines are seen,
although the most striking increase is in the erythrocyte line. Basophils and
mast cells are interrelated cell types that can both release histamine and a
variety of vasoactive chemicals and cytokines. An occasionally helpful clinical
clue is that itchiness not related to an obvious rash can be due to increased
numbers of these cell lines.
High eosinophil (choice B) counts might be associated with itchiness, but in
non-allergic diseases, the high mast cell and basophil counts are more likely
to cause symptoms.
Erythrocytes (choice C), neutrophils (choice D), and platelets (choice E) do
not contain granules that would be likely to cause itchiness.
A 62-year-old woman consults her physician because she has been having
headaches, dizziness, and tinnitus. The patient also reports that
her skin seems to be a great deal more itchy than several years ago, and that the
episodes of itchiness are sometimes triggered by a hot
shower. Physical examination demonstrates hypertension, splenomegaly, and a
ruddy complexion seen best in the face, palms, and nail
beds. The patient did not appear to be dehydrated.
Question 4 of 4
If this patient has a normal arteriaI O2 saturation level and low erythropoietin,
which of the following is the most likely diagnosis?
/ A. Chronic lung disease
/ B. Chronic myelogenous leukemia
/ C. Essential thrombocythemia
/ D. Polycythemia vera
/ E. Tumor-associated erythrocytosis
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Explanation - Q: 7.4
Close
The correct answer is D. Polycythemia vera is an idiopathic chronic
myeloproliferative disorder that is characterized by an increased red cell
mass. For the diagnosis to be formally made, the patient must have either all
major criteria for diagnosis, or the first two major criteria plus any two minor
criteria. Major criteria include (1) a red cell mass equal to or greater than 36
mL/kg in men and 32 mL/kg in women; (2) an arterial O2 saturation greater
than 92%; and (3) splenomegaly (related to extramedullary hematopoiesis).
Minor criteria include (1) thrombocytosis greater than 400 x 103/microliter;
leukocytosis greater than 12 x 103/microliter; leukocyte alkaline phosphatase
activity greater than 100 in the absence of fever and infection (this helps
exclude chronic myelogenous leukemia, choice B); and serum B12 greater
than 900 pg/mL or unsaturated B12-binding capacity greater than 2200
pg/mL (B12 abnormality is common in polycythemia vera). This condition is
closely related to both chronic myelogenous leukemia and essential
thrombocythemia (choice C), in that all cell lines produced in the marrow
tend to be increased, although in the case of polycythemia vera, the
predominant increase is in the erythrocyte line. Patients are usually treated
with recurrent phlebotomy (removal of blood as for a donation) to prevent
complications related to hyperviscosity of the blood (venous thrombosis,
stroke, hepatic portal vein thrombosis, angina pectoris, intermittent
claudication). These patients are also vulnerable to bleeding complications
(related to abnormal platelets), peptic ulcer disease (related to high
histamine levels from basophils and mast cells), and pruritus. Some cases of
polycythemia vera eventually "burn out" and the patient becomes anemic and
requires transfusions. Patients are also vulnerable to developing secondary
leukemias as either a direct complication of their disease or as a
complication of therapy.
Chronic lung disease (choice A) severe enough to cause erythrocytosis
would not show a normal O2 saturation.
Tumor-associated erythrocytosis (choice E) would show a high
erythropoietin.
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A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 1 of 6
The physician considers the possibility that the child has an environmental
illness. Which of the following is the most common environmental
illness of children in the United States?
/ A. Arsenic poisoning
/ B. Bismuth poisoning
/ C. Copper poisoning
/ D. Insecticide poisoning
/ E. Lead poisoning
Explanation - Q: 8.1
Close
The correct answer is E. Lead poisoning is thought to be the most common
environmental illness of children in this country. A high index of suspicion is
warranted, because no pathognomic symptoms exist and the physical
findings are usually negative. Symptoms that can be seen are often vaguely
neurologic (irritability, either sleeplessness or excessive lethargy, poor
appetite, headaches, vague changes in activity level). Abdominal pain with or
without vomiting can also be seen.
Arsenic poisoning (choice A) is less common than lead poisoning in this
country, and is mostly likely to be the result of either ground-water
contaminated by flowing through arsenic bearing rocks, or ingestion of rat
poison.
Bismuth (choice B) is used industrially and is not usually found in
environments to which children are exposed.
Copper (choice C) is relatively nontoxic, although it can contribute to liver
disease in patients with abnormal copper metabolism (e.g., Wilson's
disease).
Insecticide poisoning (choice D) can occur in children with some frequency
since insecticides are common in households, but this is less frequent than
lead poisoning.
A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 2 of 6
The child's family is poor, and lives in an apartment built in the 1930's and never
substantively remodeled. Rodents and insects are common
in the building, as are pesticides used to try to eliminate them. If the child does
have the most common environmental illness in US children,
which of the following would be the most likely source of the poison?
/ A. Crumbling sidewalk fragments
/ B. OId metal doorknob
/ C. OId paint chips
/ D. Rat poison
/ E. Roach poison
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Explanation - Q: 8.2
Close
The correct answer is C. In the past, the interior paint used on houses often
contained lead, which provided a very white color to the paint (to which other
colors could be added to produce vibrant paints). These paints are no longer
in use, but there is enough very old housing still present in this country that
lead poisoning remains a significant problem. The old paint often chips off
and the chips may be ingested by toddlers. The affected children have often
been bottle-fed for protracted periods and have developed some degree of
pica (ingestion of non-food substances). Lead was also commonly used in
plumbing pipes in early houses as well. In black, non-Hispanic children living
in homes built before 1946, the prevalence of lead poisoning is almost 22%.
The rates drop to 13.7% in those living in homes built from 1946 to 1973, and
then to 3.4% in homes built after 1973. Rates of lead poisoning are lower
among Mexican-American children (13%, 2.3% and 1.6%, respectively) and
white, non-Hispanic children (5.6%, 1.4%, and 1.5%, respectively). Lead
poisoning from paints also is occasionally a problem when decorative dishes
are used to contain acid foods (the classic example is an imported Mexican
pitcher used for orange juice) that leak the lead. Also, exposures can come
from some folk remedies and from adults working in lead-related
occupations.
The crumbling sidewalk fragments (choice A) and the old metal doorknob
(choice B) are probably non-toxic.
Rat poison (choice D) can contain arsenic and roach poison (choice E) can
contain organophosphate insecticides.
A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 3 of 6
Which of the following is traditionally associated with this child's disease, but is
actually now uncommonly seen?
/ A. Aphthous ulcer on inside of cheek
/ B. Dark line on the gums
/ C. Fissures on the tongue
/ D. Hairy leukoplakia of the tongue
/ E. Pyogenic granuloma of gums
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Explanation - Q: 8.3
Close
The correct answer is B. The "lead-line," which is due to lead deposition
along the gums, is traditionally associated with lead poisoning, but has
become uncommon because we are picking up most cases of lead poisoning
earlier, when the body load of lead is smaller. The physician should look for it
in suspected cases of lead poisoning, even though he or she probably won't
find it.
Aphthous ulcers (choice A), commonly called "canker sores," are idiopathic
benign oral ulcers that can occur with emotional distress, or unrelated to any
obvious inciting causes.
Fissures on the tongue (choice C) can be seen with a variety of nutritional
deficiencies.
Hairy leukoplakia (choice D) is a distinctive oral lesion (fuzzy white tongue)
seen in some AIDS patients.
Pyrogenic granuloma (choice E) produces a ball-like red mass lesion, and
can occur sporadically or in association with pregnancy.
A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 4 of 6
This child's disease often produces an anemia that on peripheral smear most
closely resembles which of the following?
/ A. Folate deficiency
/ B. Hereditary spherocytosis
/ C. Iron deficiency
/ D. Sickle cell anemia
/ E. Vitamin B12 deficiency
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Explanation - Q: 8.4
Close
The correct answer is C. The anemia of lead toxicity is a microcytic,
hypochromic anemia that closely resembles that of iron deficiency on the
peripheral blood smear. While the gold standard for diagnosing lead
poisoning is the whole blood lead level, the free erythrocyte protoporphyrin
level is often used to demonstrate the degree of biological abnormalities that
exists. Lead interferes with ferrochelatase, the enzyme helps to incorporate
iron into the protoporphyrin molecule in hemoglobin synthesis. This is why
the anemia of lead poisoning clinically resembles that of iron deficiency - the
iron is present, but the body cannot use it.
Folate deficiency (choice A) and vitamin B12 deficiency (choice E) both
produce macrocytic anemia.
In hereditary spherocytosis (choice B), all of the erythrocytes are small
spheres that lack the biconcave shape typical of normal erythrocyte.
In sickle cell anemia (choice D), even when the patient is not in a sickling
crisis, at least a few markedly deformed sickle cells will be seen.
Question 5 of 6
A peripheral blood smear is examined (above). Which of the following diagnostic
features is present in the smear?
/ A. Acanthocytes
/ B. Auer rods
/ C. Basophilic stippling
/ D. Hairy cells
/ E. Sickle cells
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Explanation - Q: 8.5
Close
The correct answer is C. Basophilic stippling causes round, dark-blue
granules in red blood cells on smears stained with brilliant cresyl blue (the
granules are more red-purple on Wright/Giemsa stains). The granules are
precipitated ribosomes and mitochondria, and are usually caused by an
impairment of maturation in the bone marrow. Causes include lead
poisoning, exposure to some drugs, severe burns, anemias, and septicemia.
Acanthocytes (choice A) are "star-like" red cells with irregularly spaced
projections that can be found in abetalipoproteinemia and some liver
diseases.
Auer rods (choice B) are elongated, bluish-red rods composed of fused
lysosomal granules. They are found in the cytoplasm of cells in the
myelocytic and monoblastic lines in patients with acute myelogenous
leukemia.
Hairy cells (choice D) are seen only in hairy cell leukemia and are white cells
with fine, irregular pseudopods ("hairs") and immature nuclear features.
Sickle cells (choice E) are seen in sickle cell anemia, not lead poisoning.
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A 3-year-child is taken to a physician for a routine physical examination. On
questioning about any problems she may have noticed that
concern her, the mother comments that the child does not seem to be feeling
well much of the time. She has noticed that the child is irritable,
sleeps poorly, and is often constipated. Physical examination is unremarkable.
Office hematocrit shows a mild anemia.
Question 6 of 6
Which of the following is an oral drug that can be used to treat this disorder in
children?
/ A. Amantadine
/ B. Succimer
/ C. Sulfasalazine
/ D. Tolbutamide
/ E. Tubocurarine
Explanation - Q: 8.6
Close
The correct answer is B. Lead poisoning is treated with lead chelating
agents to increase urinary secretion. Succimer is the only one of these
agents which has been approved for oral use in children; D-penicillamine is
also commonly used, but has never been formally approved for this use by
the FDA. Other chelating agents that are sometimes used in severe acute
lead poisoning include dimercaprol (IM) and calcium disodium edetate (IM or
IV).
Amantadine (choice A) is an antiviral agent active against influenza A virus.
Sulfasalazine (choice C) is used in rheumatoid arthritis.
Tolbutamide (choice D) is a sulfonylurea used as an oral hypoglycemic
agent.
Tubocurarine (choice E) is a skeletal muscle relaxant.
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A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 1 of 5
Which of the following is the most likely diagnosis?
/
/
/
/
/
A. Chronic myelogenous leukemia
B. Erythroleukemia
C. Hairy cell leukemia
D. Hodgkin's lymphoma
E. Metastatic oat cell carcinoma
Explanation - Q: 9.1
Close
The correct answer is C. Hairy cell leukemia is a chronic leukemia that
often has an indolent course. It can present with weakness and fatigue due
to anemia, bleeding due to thrombocytopenia, or fever and infections due to
neutropenia. Almost every patient has splenomegaly, which is massive in 4/5
of cases. The male to female ratio is 5:1 and the patients are usually middle
aged with a median age of 52. The other conditions listed would not have
"hairy cells" in the peripheral blood.
A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 2 of 5
Which of the following tests could confirm this diagnosis?
/ A. 5-hydroxyindoleacetic acid
/ B. Free erythrocyte protoporphyrin
/ C. Homovanillic acid
/ D. Myelin basic protein
/ E. Tartrate-resistant acid phosphatase
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Explanation - Q: 9.2
Close
The correct answer is E. Strong positivity for tartrate-resistant acid
phosphatase (TRAP) is a classic finding in hairy cell leukemia cells, and you
may see an item about this on a USMLE question. However, you should also
be aware that this test is no longer available at many centers.
5-hydroxyindoleacetic acid (choice A) is a serotonin derivative used in
diagnosing carcinoid tumors.
Free erythrocyte protoporphyrin (choice B) is used in the evaluation of
porphyrias.
Homovanillic acid (choice C) is a urinary catecholamine derivative that is
used in diagnosis of pheochromocytomas.
Myelin basic protein (choice D) is used in diagnosing multiple sclerosis.
A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 3 of 5
Which of the following immunologic markers is considered to have high
sensitivity and specificity for this patient's disease?
/ A. BIy-7
/ B. CD3
/ C. CD19
/ D. CD20
/ E. CD21
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Explanation - Q: 9.3
Close
The correct answer is A. Immunophenotyping of peripheral blood has
mostly replaced the (less sensitive) TRAP test for diagnosis, and is thought
to be capable of making the correct diagnosis in this setting in over 90% of
cases, even when only very small numbers of circulating leukemia cells are
present. Monoclonal Bly-7 is both sensitive and specific for hairy cell
leukemia cells.
These cells also express CD19 (choice C) and CD20 (choice D), but these
are common pan-B cell antigens and are not specific for hairy cell leukemia.
They do not express the late B cell antigen CD21 (choice E) or the T-cell
antigen CD3 (choice B). A last useful tip is that they also stain for CD22 at a
higher intensity than normal B cells.
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A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 4 of 5
The cells described are abnormal cells in which of the following cell lines?
/ A. B-Iymphocytes
/ B. Eosinophils
/ C. Macrophages
/ D. Neutrophils
/ E. T-Iymphocytes
Explanation - Q: 9.4
Close
The correct answer is A. Hairy cell leukemia cells are an abnormal type of
mature B lymphocytes. Unlike many leukemias, these cells are often present
in circulating blood in small numbers, although involvement of the spleen is
more prominent. Bone marrow aspiration is often unsuccessful due to a dry
tap; core biopsy will show the tumor cells with abundant cytoplasm infiltrating
a fine fibrillar network. The hairy cell leukemia cells are sensitive to purine
analogues (cladribine) and interferons. Granulocyte colony stimulating factor
(filgrastim) is used to reduce the neutropenia that may produce lifethreatening susceptibility to infections (gram-negative rods, atypical
mycobacteria, disseminated fungus, and Pneumocystis). Patients often
experience long-term remissions with chemotherapy, and late relapses (after
5-10 years) often respond a second time to the same chemotherapy. The
other choices listed are distracters.
A 55-year-old man consults a physician because he has been experiencing
chronic weakness and fatigue. Physical examination is notable
for the presence of petechiae and a massively enlarged spleen. A peripheral
blood smear was sent, which on review demonstrates anemia,
thrombocytopenia, modest neutropenia, and the presence of small numbers of
unusual white blood cells. These blood cells are small cells
with a moderate amount of cytoplasm that are covered with distinctive
cytoplasmic projections that appear to be long microvilli.
Question 5 of 5
Which of the following is the most appropriate pharmacotherapy?
/ A. CIadribine
/ B. Cyclophosphamide
/ C. FIudarabine
/ D. Methotrexate
/ E. 6-Mercaptopurine
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Explanation - Q: 9.5
Close
The correct answer is A. The treatment of hairy cell leukemia (HCL) has
dramatically improved with the development of newer, more effective
antineoplastic agents, such as cladribine. Cladribine is considered to be the
treatment of choice for this condition. This is a relatively nontoxic drug that
provides benefit in approximately 95% of all cases and a complete remission
in more than 80% of these cases. In general, response rates are long-lasting,
with few patients relapsing in the first several years. Interferon and
splenectomy are rarely used today to treat this condition. With respect to the
pharmacology of this agent, cladribine is indicated for the treatment of active
HCL as defined by clinically significant anemia, neutropenia,
thrombocytopenia, or disease related symptoms. Non-labeled uses include
treatment of advanced cutaneous T-cell lymphoma, chronic lymphocytic
leukemia, non-Hodgkin lymphomas, acute myeloid leukemia, and
autoimmune hemolytic anemia.
Cyclophosphamide (choice B) is an antineoplastic commonly used in the
treatment of a variety of malignant diseases, such as Hodgkin disease as
well as treatment of several types of leukemia. This medication is an
alkylating agent related to the nitrogen mustards. It suppresses cell-mediated
and humoral immunity as to interfere with "recall responses", causes
lymphopenia of B and T cells, and reduces serum immunoglobulin levels.
Fludarabine (choice C) is an antimetabolite antineoplastic agent indicated
for the treatment of chronic lymphocytic leukemia (CLL). Although not
approved for hairy cell leukemia, this agent has been used with some
success in the treatment of this condition. Fludarabine is specifically a
fluorinated nucleotide analog of the antiviral agent, vidarabine. This agent
appears act by inhibiting DNA polymerase alpha, ribonucleotide reductase,
and DNA primase, thus inhibiting DNA synthesis.
Methotrexate (choice D) is an agent that competitively inhibits dihydrofolic
acid reductase and is also used in antineoplastic therapeutic treatment
regimens, such as ALL, cancers of the head, neck, and breast, usually in
combination with other therapies. It is also used to treat severe, active,
classic or definite rheumatoid arthritis in adults who have an insufficient
response with conventional therapies, and for severe, recalcitrant, disabling
psoriasis.
6-Mercaptopurine (choice E), otherwise known as mercaptopurine or 6-MP,
is a purine analog antimetabolite indicated for the treatment of remission
induction and maintenance therapy of acute lymphocytic leukemia (ALL) as
well as treatment of acute myelogenous (and acute myelomonocytic)
leukemia.