A 25-year-old black schoolteacher was brought to the emergency room because of a six- hour history of severe pain in the

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A 25-year-old black schoolteacher was brought to the emergency
room because of a six- hour history of severe pain in the back,
knees and ankles. She has had occasional attacks of this type in
the past. A brother with similar episodes allegedly had a positive
sickle preparation. Her parents were entirely well. Physical
examination revealed that she was slightly jaundiced and pale, and
had a spleen palpable 4 cm below the left costal margin. Lab Data:
Hgb = 9.9 g/dL; RBC = 3.2 million / ML; HCT = 30%; WBCs = 12,000/
uL with normal differential; Retics = 13%; serum bilirubin = 3.2
mg/dL of unconjugated bilirubin. The patient recovered after 48
hours and returned to work. Two years later, her hematocrit was
noted to be gradually falling to a level of 23%, with a decrease in
her Retic count from her usual 18-20% down to 4%. A peripheral
smear revealed the target cells and sickled cells as before, but
also an increase in hypersegmented polys and some oval macrocytes.
A bone marrow aspirate was performed. 5. Predict the findings on
the bone marrow and explain the probable cause of the falling
hematocrit. The patient was treated appropriately and her
hematocrit returned to its previous level of 30%. Six months later,
the patient was readmitted five days after the onset of fever,
muscle aches and malaise during a flu-like illness. Her hematocrit,
which had been 30% one week before, was now 18% with a retic count
of 0.5%.
6. How do you explain the fall in hematocrit and reticulocyte
count? The patient did well over the next five years, with only an
occasional painful crisis. She was then admitted because of dyspnea
on exertion and ankle swelling. Her physician determined that she
was in congestive heart failure.
7. What factors contributed to the heart failure in this
patient?
8. Describe the pathophysiology of the anemia in SS, SC, and CC
diseases.
9. Review and list the clinical problems encountered in patients
with aggregating hemoglobins. Discuss the pathophysiology of each
complication.