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A 1 8-year-old female patien t of I ranian descent presen ts to the emergency room with back and leg pain. Laboratory results reveal leukocyte count of 1 7 , LJOO/,uL. hemoglo-bin of 1 1 . 5 g/d L, MCV of 65 FL. and platelet count of 2 97.000/,uL. Peripheral smear is shown below.
Which of the following choices represents the expected pattern on hemoglobin electrophoresis for this condition?
- HbS 90% HbF S% HbA2 <3.5%
- HbC 50% HbS 45% HbF 2.5% HbA2 2.5%
- HbA 60% HbS 35% HbF <2% HbA2 < 3.5%
- HbS 60% HbA 30% HbF 3% HbA2 7%
Medicine
D. Choice A represents sickle cell disease (SS), choice B represents sickle-HbC disease (SC), choice C represents sickle-cell trait, and choice D represents sickle-beta(+) thalassemia. The peripheral smear shows target cells, which are commonly seen in thalassemia, and a typical drepanocyte (sickle cell). HbC is seen in patients of African descent, whereas thalassemias are associated with Mediterra-nean populations. In addition, an MCV of 65 f.!L favors sickle-beta thalassemia. In sickle cell anemia and trait, the MCV is normal, and in SC disease, the MCV may be normal or mildly microcytic.
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