Nonspherocytic Haemolytic Anaemia and Severe Jaundice in a Newborn With Partial Pyruvate Kinase Deficiency

Summary We present a case of familial non spherocytic haemolytic anaemia which began with a severe neonatal jaundice (I. B. 28 mg/100 ml). The propositus presented a partial PK deficiency, which was also present in the father but not in the mother. We were able to exclude other conditions which can be responsible for haemolytic anaemia, and neonatal jaundice. The hypothesis that also a partial PK deficiency can, in particular conditions, be responsible for a severe haemolytic process, is suggested.