Clinical spectrum and severity of hemolytic anemia in glucose 6-phosphate dehydrogenase-deficient children receiving dapsone
Drug-induced acute hemolytic anemia (AHA) led to the discovery of G6PD deficiency. However, most clinical data are from isolated case reports. In two clinical trials of antimalarial preparations containing dapsone (4,4'-diaminodiphenylsulfone; 2.5 mg/kg once daily for three days), 95 G6PD-deficient hemizygous boys, 24 G6PD-deficient homozygous girls and 200 girls heterozygous for G6PD deficiency received this agent. In the first two groups, there was a maximum decrease in hemoglobin averaging -2.64 g/dL (range -6.70 to +0.30 g/dL); significantly greater than for the comparator group receiving artemether-lumefantrine (adjusted difference -1.46 g/dL; 95% confidence interval -1.76, -1.15). Hemoglobin concentrations were decreased by ≥40% versus pre-treatment in 24/119 (20.2%) of the G6PD-deficient children; 13/119 (10.9%) required blood transfusion. In the heterozygous girls, the mean maximum decrease in hemoglobin was -1.83 g/dL (range +0.90 to -5.20 g/dL); 1/200 (0.5%) required blood transfusion. All children eventually recovered. All the G6PD-deficient children had the G6PD A- variant, i.e. mutations V68M and N126D. Drug-induced AHA in G6PD A- subjects can be life-threatening, depending on the nature and dosage of the drug trigger. Therefore, contrary to current perception, in clinical terms the A- type of G6PD deficiency cannot be regarded as mild.
Pamba, A.; Richardson, N.D.; Carter, N.; Duparc, S.; Premji, Z.; Tiono, A.B.; Luzzatto, L. Clinical spectrum and severity of hemolytic anemia in glucose 6-phosphate dehydrogenase-deficient children receiving dapsone. Blood (2012) : [DOI: 10.1182/blood-2012-03-416032] Published online 19/9/2012