Hereditary Spherocytosis

Department of Haematology

Osmotic fragility test no longer performed. See BSCH guidelines for diagnosis and management of hereditary spherocytosis
Osmotic fragility test is no longer recommended due to lack of both sensitivity (normal results may occur in 10-20% of HS) and specificity (unable to differentiate between immune and non-immune causes of spherocytosis).
Eosin-5-maleimide (EMA) binding dye test by flow cytometry is now favoured as a screening test (sensitivity 92.7%, specificity 99.1%)
Samples are referred to the Sheffield Children’s Hospital

1 week

Hereditary Spherocytosis flow cytometry test ratio 0.85-1.30