Q:

What is the pathophysiologic difference between hereditary spherocytosis (HS) and HE?

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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?


  1. HE involves vertical protein interactions, most commonly affecting ankyrin
  2. HE involves horizontal protein interactions, most commonly affecting spectrin
  3. HS involves horizontal protein interactions, most commonly affecting spectrin
  4. HS involves vertical protein interactions, most commonly affecting band 3

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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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