Effect of Desferrioxamine in Acute Haemolytic Anaemia of Glucose-6-Phosphate Dehydrogenase Deficiency

Abstract

The effectiveness of desferrioxamine (DFO) in ameliorating the severity of the acute haemolysis of glucose-6-phosphate dehydrogenase (G6PD) deficiency was studied in 167 children with G6PD deficiency during an acute haemolytic crisis. All patients received packed cell transfusion on admission if their Hb levels were <8 g/dl, which was repeated as needed. Eighty patients also received a single dose of DFO 30–40 mg/kg by slow intravenous infusion (DFO group). The remaining 87 children did not receive DFO (control group). The need for more than one transfusion was less frequent in the DFO group as compared to the control group (p = 0.01). The need for late transfusion (transfusion after 36 h of admission) was also less in the DFO group (7%) compared to 21% in the control group (p = 0.02). On average, children in the DFO group needed less packed red blood cells (16.5 ml/kg body weight) than the control group (22.8 ml/kg body weight) and the difference was highly significant (p = 0.0001). We conclude from this study that DFO in a small dose is effective in the treatment of acute haemolytic crises of G6PD deficiency. It shortens the duration of the crisis and decreases the amount of blood transfusion needed.

Glucose-6-Phosphate Dehydrogenase Deficiency and Antimalarial Drug Development

“…Desferrioxamine has been used during acute hemolytic crisis in children and has been claimed to shorten the duration of the crisis and decrease the frequency of blood transfusion needed. 111,112 Early detection of hemolysis. Early indicators of G6PD deficiency-related hemolysis would be valuable for use in clinical trials both from a safety perspective and as outcomes investigating hemolytic potential.…”

COVID-19 Drugs and Glucose-6-phosphate Dehydrogenase Deficiency: A Matter of Life Threatening And Public Health

“…ABCMED 9(4): [24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40] general population, to better evaluate their safety and possible alternatives.…”

“…A significant decrease of H 2 O 2 -induced oxidative hemolysis was observed in erythrocytes cells with deferoxamine administration in vitro, as iron enhances ROS generation [31]. Deferoxamine treatment reduced blood transfusion necessity and shortened the clinical course of acute hemolytic crisis in all the G6PD deficient patients (80 out of 167 children) that received a single dose of deferoxamine (30-40 mg/kg IV) [32]. Deferoxamine is associated with very rare adverse reactions, such as low blood cell counts, and rare blood abnormalities [14].…”

“…Moreover, NAC acts as an indirect antioxidant because increases manganese superoxide dismutase activity in vivo, thereby protecting cells from oxidative stress-mediated toxicity [57]. Lead-induced oxidative stress was attenuated by NAC treatment in erythrocytes in vivo increasing GSH levels, and in vitro facilitating ROS detoxification in G6PD deficient hu-ABCMED 9(4): [24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39][40] man erythrocytes, effects confirmed in a randomized controlled trial that demonstrated stimulation by NAC of GSH synthesis and erythrocyte G6PD activity in 122 healthy male workers taking 200-800 mg of NAC (out of 171 total individuals), more significantly compared with a control group [66]. As described in a case report, a treatment with three doses of 10 g over 24 h of IV NAC, followed by a single week discontinuation and one-week treatment with 600 mg every 12 h, was reported to give benefit to a G6PD-deficient 44-year-old male patient with severe COVID-19 infection, which had received a single dose of HCQ (400 mg).…”