ABSTRACT
Hereditary spherocytosis is a congenital hemolytic anemia that has been a longstanding puzzle to hematologists and geneticists (see recent reviews [1–3]). It is a common disorder affecting ~1 in 5000 individuals of Northern European ancestry [4]. The genetic defect is incompletely defined but results in erythrocytes that are abnormally fragile and become increasingly spherical rather than retaining the normal biconcave disk shape.
