Acute massive haemolysis in children with glucose-6-phosphate dehydrogenase deficiency

HKY Lau, CH Li, ACW Lee
Department of Paediatrics and Adolescent Medicine, Tuen Mun Hospital, Tuen Mun, Hong Kong

We report seven consecutive episodes of acute massive haemolysis accompanied by symptomatic anaemia and gross haemoglobinuria in six boys with glucose-6-phosphate dehydrogenase deficiency seen in a regional hospital during a 12-year period. They presented at a mean age of 5.5 years (range, 1.5-11.3 years) with trough haemoglobin levels between 35 and 84 g/L. Two children developed transient renal impairment. Five children required erythrocyte transfusion, of whom one underwent exchange transfusion during the oliguric phase. Three patients required intensive care but all recovered from the haemolysis. The probable precipitating factors included consumption of fava beans (n=2), exposure to mothballs (n=1), treatment with herbal medicine or intramuscular injection of unknown nature (n=3), and upper respiratory tract infection (n=1). Although uncommon, acute massive haemolysis remains a life-threatening complication in children with glucose-6-phosphate dehydrogenase deficiency. Improvement in patient education and public health measures is suggested.

Hong Kong Med J 2006;12:149-51

Key words: Favism; Glucosephosphate dehydrogenase deficiency; Hemolysis; Naphthalenes; Neonatal screening