Ankyrin deficiency is the most common defect in dominant and non dominant hereditary spherocytosis.

Abstract

We investigated RBC protein abnormalities in 14 HS familieswith clear autosomal dominant inheritance patterns (32patients) and 6 additional patients belonging to families inwhich the propositus was the only one affected. We could notestablish whether recessive inheritance or a new dominantmutation was present, so we referred to them as