A S LJ-year-old male patien t is being evaluated for a lung mass seen on a CT scan for chest pain. He is otherwise healthy and his physical exam is normal. Complete blood count shows leukocyte count 5 . 700/jl.l. hemoglobin 1 LJ .2 g/d l. and platelet count 2 89.000/jl.L. The peripheral blood smear is shown below.

What is the pathophysiologic difference between hereditary spherocytosis (HS) and HE?
- HE involves vertical protein interactions, most commonly affecting ankyrin
- HE involves horizontal protein interactions, most commonly affecting spectrin
- HS involves horizontal protein interactions, most commonly affecting spectrin
- HS involves vertical protein interactions, most commonly affecting band 3
B. The principal defect in HE is a mechanical weakness or fragility of the erythrocyte mem-brane, primarily involving horizontal protein interactions, especially spectrin-spectrin and spectrin-protein 4.1 and the lipid bilayer. On the other hand, HS involves abnormalities of vertical protein interactions, especially ankyrin and ankyrin-spectrin.
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