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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