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Effect of desferrioxamine B on hemolysis in glucose-6-phosphate dehydrogenase deficiency
A S Khalifa1, M S el-Alfy, G Mokhtar
1Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Insights
Desferrioxamine B aids in managing hemolytic crises for infants and children with glucose-6-phosphate dehydrogenase (G6PD) deficiency. This treatment, alongside blood transfusions, shows significant improvements in hemoglobin levels and reduced hemoglobinuria.
Area of Science:
- Pediatrics
- Hematology
- Pharmacology
Background:
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common inherited disorder that can lead to hemolytic crises.
- Hemolytic crises in G6PD-deficient patients necessitate effective management strategies to prevent complications.
Purpose of the Study:
- To evaluate the efficacy of desferrioxamine B as an adjunct to packed red cell transfusion in managing hemolytic crisis in pediatric G6PD-deficient patients.
- To assess the impact of desferrioxamine B on hemoglobin levels, reticulocyte count, and hemoglobinuria.
Main Methods:
- A study involving 24 infants and children (3-36 months) with G6PD deficiency during hemolytic crisis.
- Patients were divided into two groups: one receiving desferrioxamine B plus packed red cell transfusion, and a control group receiving only transfusion.
- Sequential monitoring of hemoglobin, reticulocyte count, and hemoglobinuria at various time points post-treatment.
Main Results:
- The desferrioxamine B group demonstrated significantly higher hemoglobin levels at 48 and 72 hours post-treatment (p < 0.01).
- Hemoglobinuria resolved in 78.5% of patients treated with desferrioxamine B, compared to only 30% in the transfusion-only group by 72 hours.
- While not explicitly detailed, reticulocyte counts were also monitored sequentially.
Conclusions:
- Desferrioxamine B is effective in shortening the duration of hemolytic crisis in pediatric G6PD-deficient patients.
- Desferrioxamine B can be utilized as an adjuvant therapy alongside packed red cell transfusions for improved outcomes.
Abstract:
Twenty-four infants and children suffering from glucose-6-phosphate dehydrogenase (G6PD) deficiency during hemolytic crisis were included in this study. Their ages ranged between 3 and 36 months with a median of 10 months. 22 were males and 2 were females. Fourteen out of them received a single bolus dose of desferrioxamine B 500 mg intravenously followed by packed red cell transfusion, while the remaining 10 cases were only transfused. Sequential estimation of hemoglobin level, reticulocytic count and hemoglobinuria was done before treatment, 3, 24, 48 and 72 h thereafter. The hemoglobin level was higher in the desferrioxamine B group. The degree of increase was statistically significant at 48 and 72 h (p less than 0.01). Hemoglobinuria stopped in 78.5% in the first group and only in 30% of the second group at 72 h. It was concluded that desferrioxamine B is helpful in shortening the course of hemolytic crisis in G6PD-deficient patients. It could be used as an adjuvant to packed red cell transfusion.
