Inherited blood disorder characterized by oval or elliptical red blood cells. Usually autosomal dominant inheritance. Most common in people of African or Mediterranean descent. Spectrum from asymptomatic carriers to severe hemolytic anemia.

• 1Peripheral blood smear
• 2Osmotic fragility test
• 3Eosin-5-maleimide (EMA) binding test
• 4Genetic testing
• 5Complete blood count

Excellent for most patients. 90% are asymptomatic and have normal life expectancy. Those with compensated hemolysis may have mild anemia but normal activity levels. Severe cases (hereditary pyropoikilocytosis) may require splenectomy with good outcomes. Neonatal period may have severe hemolysis requiring phototherapy.

• Genetic counseling
• Family screening
• Prenatal diagnosis (severe variants)