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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.
The clinical severity of sickle-beta(+) thalassemia depends mostly on which of the following?
- Level of HbA, which varies based on the type of mutation in beta globin chain
- Level of HbF, which varies based on the specific sickle-cell mutation phenotype
- Level of HbA2, which is dependent on the type of mutation in alpha chain
- Level ofHbS, which varies based on the specific sickle-cell mutation phenotype
Medicine
A. The level of HbA determines the disease severity in patients with sickle-beta(+) thalas-semia; the higher the level of Hgb A, the milder the disease (Br J Haematol. 199 1;77(3):386-9 1).
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