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A 3LJ-year-old male patien t is seen in hematology clinic to establish care. He sta tes he has sickle cell disease. Complete blood count shows leu kocyte count of 5.300/,ul. hemoglobin 1 2 .3 g/d l. MCV 77 fl. and platelet count 212 . 000/,ul. The peripheral smear is shown below.
Hemoglobin electrophoresis is performed and shows equal portions of HbS and HbC, consistent with HbSC disease. Which of the following statements describes HbSC disease?
- HbSC patients rarely have functional asplenia
- HbSC patients experience the same spectrum of complications as HbSS patients, but at a decreased frequency
- HbSC patients have a similar life expectancy to HbSS patients
- Patients with HbSC disease have more treatment options than HbSS patients
Medicine
B. Functional asplenia commonly occurs in HbSC patients, but at an advanced age com-pared with HbSS patients. HbSC patients have a lower incidence of the complications associated with HbSS disease. Life expectancy with SC is roughly 20 years longer than with SS. There are no treatment options for patients with SC that are unavailable to patients with SS disease (! Clin Epidemiol.
1 992;45(8):893, N Engl J Med. 1994;330(23):1639).
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