Congenital spherocytosis is often not hereditary

A retrospective study of 50 patients with hereditary spherocytosis (HS) from 41 families seen between 1967 and 1979 at the Royal Manchester Children's Hospital, and Booth Hall Children's Hospital, Manchester, England is described. Nineteen patients (38%) had no family history of spherocytosis and in 12 patients (24%), tests on the family showed no evidence of disease. Various modes of inheritance are discussed in the light of these results. The traditional name for the condition (hereditary spherocytosis) is considered unsuitable as it is clearly inaccurate in many cases. The name congenital spherocytosis would be more appropriate.