Internal-Medicine

106 5: Hematology
36. A 4-month-old baby has developed feeding
problems and diarrhea. Laboratory evaluation
reveals severe anemia with hemoglobin
of 7 g/dL. The blood film shows hypochromic
microcytic cells and target cells. In this disorder,
decreased beta-chain production leads to
excess alpha-chain production and destruction
of red cell precursors. (SELECT ONE)
37. A 4-year-old boy has a chronic microcytic
anemia, but no other symptoms. His physical
examination is normal except for splenomegaly.
His condition is due to decreased alpha-chain
production, which leads to four beta-chain
tetramer formation (HbH). (SELECT ONE)
38. A 27-year-old woman complains of constant
back pain. She recently had an episode of
severe chest and back pain that required narcotic
medications, blood transfusions, and lots
of fluids to treat. On examination, she is afebrile,
with midthoracic tenderness on palpation.
X-rays of the spine reveal periosteal reaction of
the eighth thoracic vertebra She has a chronic
blood disorder where bone infarction can occur
that may be difficult to distinguish from
osteomyelitis. (SELECT ONE)
Questions 39 through 41
(A) loss of Achillis tendon reflex
(B) increased perioperative morbidity
(C) renal concentration defect
(D) helmet cells and schistocytes are
common
(E) occurs most commonly in the first
trimester
(F) renal papillary necrosis
(G) cardiac arrhythmias
(H) increased HbH
(I) responds to folic acid
(J) paresthesias
For each patient with a hematologic disorder,
select the most likely complications or associated
conditions.
39. A 63-year-old woman with lymphoma has
been treated with vincristine for many months.
She has now developed dose-limiting side
effects from this drug. (SELECT TWO)
40. A 19-year-old woman is positive for sickle cell
disease. (SELECT TWO)
41. A 24-year-old pregnant woman is found to be
anemic. (SELECT ONE)
DIRECTIONS (Questions 42 through 59): Each of
the numbered items in this section is followed by
answers. Select the ONE lettered answer that is
BEST in each case.
42. A 32-year-old woman has had a previous child
with beta-thalassemia. She has just been confirmed
as being 6 weeks pregnant. She is worried
that the current pregnancy may also be
affected. Which of the following methods is
most likely to establish an accurate prenatal
diagnosis?
(A)
(B)
(C)
(D)
(E)
fetal ultrasound at 12 weeks
cord blood electrophoresis
chorionic villus sampling
buccal mucosal cytology of both parents
electrophoresis of amniotic fluid
43. A 27-year-old female presents with easy fatigue
and light-headedness. She also has a dry cough
and fever for the past few days. On examination,
she is pale, her lungs are clear, and the rest
is normal. A chest x-ray (CXR) shows patchy
bilateral infiltrates; the hemoglobin is 8.4 g/dL,
reticulocyte count of 6%, and many spherocytes
on the peripheral blood film. Which of the
following is the most likely significance of the
spherocytosis on the blood film?
(A) multiple long bone fracture
(B) hereditary elliptocytosis
(C) Coombs’-positive hemolytic anemia
(D) glucose-6-phosphate dehydrogenase
(G6PD) deficiency
(E) leukemia
44. A 25-year-old woman is pregnant for the first
time. At prenatal assessment, she is found to
have blood group A and Rh(D)-negative. Her
husband is tested and he is Rh(D)-positive.