Hereditary Spherocytosis
Signs & Symptoms
Anemia at any age, fluctuating jaundice, splenomegaly, pigment gallstones and aplastic crises.
Risk Factors
Women, infants and children, vegetarians
Causes
Caused by defects in the proteins involved with the vertical interactions between the membrane skeleton and the lipid bilayer of the red cell. Autosomal dominant inheritance with variable expression.
Peripheral blood characteristics
Anemia with increased reticulocytes and microspherocytes.
Important investigations & related results
Increased osmotic fragility.
Treatment & management
Splenectomy.
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