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Retinopathy
3%
1/38
Aplastic anemia with parvovirus B19 infection
26%
10/38
Stroke
11%
4/38
Gallstones
55%
21/38
Avascular necrosis of the femoral head
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The patient in this vignette most likely has hereditary spherocytosis. The increased liver excretion of bilirubin secondary to chronic RBC degradation can result in calcium bilirubinate stones. Hereditary spherocytosis is an autosomal dominant, intrinsic hemolytic anemia often caused by a mutation in spectrin, a red blood cell membrane protein. As a result, red blood cells are spherical instead of discoid in shape and have a reduced surface area to volume ratio. This condition can be diagnosed with the osmotic fragility test, which relies on these cells' increased lysis in hypotonic solutions because of their altered membrane properties. Dhaliwal et al. describe the characteristics of various types of hemolytic anemia. Hereditary spherocytosis usually presents as mild compensated anemia with an elevated MCHC. Most patients have little to no symptoms, and treatment is not warranted. However, complete or partial splenectomy may be indicated in severe cases where Hgb < 11 g/dL as well as when jaundice is present. Da Costa et al. review red blood cell membrane disorders, including hereditary spherocytosis. The RBC membrane is composed of a lipid bilayer that is linked to a spectrin skeleton. This spectrin skeleton is responsible for the flexibility and stability of the RBC membrane. Decreased membrane stability results when the lipid bilayer is not securely linked to the underlying skeleton (spectrin mutation/defect), leading to a loss of surface area as is seen in hereditary spherocytosis. Illustration A shows an example of calcium bilirubinate gallstones, which are the result of increased liver excretion of bilirubin secondary to chronic RBC degradation. Note the black color. Incorrect Answers: Answers 1, 3 ,5: These are all complications associated with sickle cell anemia but are less likely in hereditary spherocytosis. Answer 2: Aplastic anemia with parvovirus B19 infection is a possible complication with hereditary spherocytosis, however, it is less common than gallstone formation.
3.6
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