Hereditary Spherocytosis

Autosomal dominant inheritance is how hereditary spherocytosis is transmitted. The red blood cells are spherical due to a defect in a membrane cytoskeletal protein. Its incidence is from 1:1000 to 1:4500.

Clinical manifestations include hemolytic anemia, jaundice, splenomegaly, pigmented gallstones, compensatory erythroid hyperplasia, and chronic leg ulcers.

Laboratory features include spherocyte, MCV normal or slightly decreased, increased MCHC, and increased osmotic fragility indicating spheroidicity.

Treatment entails splenectomy in moderate to severe hemolysis