Clinical spectrum and severity of hemolytic anemia in glucose 6-phosphate dehydrogenase–deficient children receiving dapsone

Abstract

Drug-induced acute hemolytic anemia led to the discovery of G6PD deficiency. However, most clinical data are from isolated case reports. In 2 clinical trials of antimalarial preparations containing dapsone (4,4-diaminodiphenylsulfone; 2.5 mg/kg once daily for 3 days), 95 G6PD-deficient hemizygous boys, 24 G6PD-deficient homozygous girls, and 200 girls heterozygous for G6PD deficiency received this agent. In the first 2 groups, there was a maximum decrease in hemoglobin averaging ؊2.64 g/dL (range ؊6.70 to ؉0.30 g/dL), which was significantly greater than for the comparator group receiving artemether-lumefantrine (adjusted difference ؊1.46 g/dL; 95% confidence interval ؊1.76, ؊1.15).

Validation of the rapid test Carestart(tm) G6PD among malaria vivax-infected subjects in the Brazilian Amazon

“…The slight difference in the results of the two studies may be related to the techniques used for the quantitative analysis, which is very sensitive to small changes in the protocol (12) (30) . On the basis of this fi nding in non-malarial participants (excluding all individuals with activity ≤1IU/gHb), it was possible to calculate the normal range of enzyme activity for the local male population, namely, 3.9 to 9.8 IU/gHb.…”

“…It would similarly be important before starting treatment of hanseniasis with dapsone, since hanseniasis is an endemic disease in the Brazilian Amazon and dapsone is known to have hemolytic effects in G6PDd carriers (30) .…”

“…The mean enzymatic activity was estimated in the population of non-malarial participants, with the exclusion of participants with <1% enzymatic activity (<1.0IU/gHb) (30) (n=352). The mean activity estimated was 6.6IU/gHb and the range of normal enzyme activity (between 60% and 150% activity) was estimated to be between 3.9and 9.8IU/gHb.…”

G6PD Deficiency in an HIV Clinic Setting in the Dominican Republic

“…We could not determine whether clinic-prescribed medications, such as TMP-SMX and dapsone, caused hemolysis, which requires a prospective study; however, these can precipitate severe hemolysis in G6PD deficiency. 24,[31][32][33][34] Anemia in HIV-infected patients is often multifactorial, complicating the identification of G6PD deficiency. Although laboratory tests for hemolysis are helpful, they are rarely performed in low-resource settings.…”

“…Class III variants (10-60% activity) are most prevalent and individuals of African ancestry with the G6PD A− variant can experience life-threatening drug-induced hemolysis. 24 Although G6PD A− was expected to predominate in the Dominican Republic, to avoid missing unusual variants, 25,26 we used a comprehensive sequencing strategy.…”