Abstract
Hereditary spherocytosis is a hemolytic disease transmitted by autosomal dominant trait. The jaundice of the patients with HS in the neonatal period often becomes severe. Then patients with HS often have past history of blood exchange transfusion because of severe jaundice in the neonatal period.
We experienced a patient who was suspected to have HS soon after birth for her early jaundice and her family history, and blood exchange transfusion was done. The patient was a first born and the product of a 38 weeks gestational normal delivery weighing 2, 990g. The patient's mother, maternal grandfather and maternal aunt have HS and her mother and grandfather have past history of splenectomy.
Nausea and vomiting began at 17 hours after birth and jaundice on the skin appeared at 22 hours after birth. Serum total bilirubin level was 12mg/dl at 31 hours after birth and blood exchange transfusion was done immediately. Jaundice gradually disappeared and the patient's condition was favorable after that. But severe anemia was noticed at 40 days after birth. The value of hemoglobin was 7.6g/dl and 100ml of blood was transfused to the patient.
Moderate amount of spherocytes were seen in the mother's peripheral blood. On the contrary, spherocytes were scarcely seen in the patient's peripheral blood. When auto-hemolytic test was done at 3-months-old, 8.29% of the patient's red blood cells incubated for 48 hours without glucose hemolyzed and the red blood cells incubated with glucose became spherocytec. Osmotic fragility test of the patient's red blood cell showed normal. This result may be because of the influence of blood transfusion. Other hemolytic screening tests such as hemoglobin electrophoresis and Heinz body formation test were normal.
The cause of HS is considered to be decreased lipid on HS erythrocyte membrane. Severe hemolysis of HS newborns may be related to hypolipidemia in neonatal period. The value of total cholesterol of the patient's serum before blood exchange transfusion was 57mg/dl.
