Original Paper Acta Haematol 1999;101:145–148 Effect of Desferrioxamine in Acute Haemolytic Anaemia of Glucose-6-Phosphate Dehydrogenase Deficiency H.S. Al-Rimawi a M. Al-Sheyyab a A. Batieha b H. El-Shanti a,c F. Abuekteish a Departments of a Paediatrics, b Community Medicine and c Medical Technology, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan Received: January 28, 1998 Accepted after revision: October 9, 1998 Dr. Hala Saleh Al-Rimawi Assistant Professor of Paediatrics Jordan University of Science and Technology PO Box 3030, Irbid (Jordan) Fax +962 02 295123 ABC Fax + 41 61 306 12 34 E-Mail karger@karger. ch www.karger.com © 1999 S. Karger AG, Basel 0001–5792/99/1013–0145$17.50/0 Accessible online at: http://BioMedNet.com/ karger Key Words Acute haemolytic anaemia W Desferrioxamine W Favism W Glucose-6-phosphate dehydrogenase deficiency Abstract The effectiveness of desferrioxamine (DFO) in ameliorat- ing the severity of the acute haemolysis of glucose-6- phosphate dehydrogenase (G6PD) deficiency was stud- ied in 167 children with G6PD deficiency during an acute haemolytic crisis. All patients received packed cell trans- fusion 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 com- pared 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. Introduction Glucose-6-phosphate dehydrogenase (EC 1.1.49; G6PD) is the enzyme that catalyzes the first step in a chain of reactions in the pentose phosphate pathway. In the red cells, it is the only source of NADPH required for the reduction of oxidized glutathion [1]. Deficiency of G6PD leads to susceptibility to oxidative stress when exposed to certain drugs or chemicals [1]. G6PD deficien- cy is an X-linked hereditary disease. The overall reported prevalence in northern Jordan ranged from 4.6 to 5.5% [2]. Oxidative stress in the form of free radical production, which is catalyzed by transitional metal ions such as iron, plays an important role in the formation of methaemoglo- bin and the peroxidation of membrane lipids leading to red cell membrane destruction and haemolysis [1, 3]. The iron-chelating agent, desferrioxamine (DFO), can arrest red cell haemolysis through inhibition of membrane- mediated iron-induced free radical production [4], thus