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Hereditary spherocytosis is a genetic blood disorder that affects the membrane of red blood cells, causing them to become round and sphere-shaped instead of their normal flexible, disc-like form. These abnormal cells are less durable and are destroyed prematurely by the spleen, leading to chronic haemolytic anaemia. The condition is most commonly inherited in an autosomal dominant pattern, although autosomal recessive cases also occur.

Symptoms vary from mild to severe and may include fatigue, pale skin, jaundice, enlarged spleen, and the formation of gallstones due to increased breakdown of red blood cells. Children with severe disease may experience delayed growth, while some individuals remain largely symptom-free throughout life. Episodes of worsening anaemia can occur during infections or following aplastic crises.

Diagnosis involves a complete blood count (CBC), peripheral blood smear, reticulocyte count, bilirubin testing, and specialised tests such as the eosin-5-maleimide (EMA) binding test or osmotic fragility test. Family history and genetic testing may also support the diagnosis.

In moderate to severe cases, splenectomy may reduce red blood cell destruction, although appropriate vaccinations and infection prevention measures are essential. With timely diagnosis and ongoing care, most individuals achieve an excellent long-term prognosis.