✅ Hereditary spherocytosis (HS) belongs to the group of congenital hemolytic anemias resulting from plasma membrane protein deficiency.
✅ The clinical presentation of HS is highly variable and is classified according to clinical severity (mild, moderate and severe) based on hemoglobin (Hb), reticulocyte % and serum bilirubin levels.
✅ Symptoms are primarily related to anemia, though complications of hemolysis may occur, including splenomegaly, pigment gallstones, and iron overload.
✅ Diagnosis of HS is suspected in a patient with Coombs-negative (non-immune) hemolytic anemia, presence of spherocytes on the peripheral blood smear, increased mean corpuscular hemoglobin concentration (MCHC) and a positive family history of HS.
✅ Diagnosis of HS is confirmed using an EMA binding assay or osmotic fragility test.
✅ Treatment is supportive and includes folic acid supplements, red cell transfusion, splenectomy (in severe cases) and iron chelation.