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.

Keywords:
Acute haemolytic anaemia, Desferrioxamine, Favism, Glucose-6-phosphate dehydrogenase deficiency
1.
Piomelli S: G6PD deficiency and related disorders of the pentose pathway; in Nathan D, Oski F (eds): Haematology of Infancy and Childhood. London, Saunders, 1987, vol 1, pp 584–612.
2.
Sharif L, Bashir N, Barkawi M, Sheyab M: Glucose-6-phosphate dehydrogenase isozymes in Jordan valley. Ann Saudi Med 1993;13:269–271.
3.
Kosower NS, Zipser Y, Faltin Z: Membrane thiol-disulfide status in glucose-6-phosphate dehydrogenase deficient red cells. Biochim Biophys Acta 1982;691:345.
4.
Rice-Evance C, Baysal E, Kontoghiorghes GJ, Flynn DM, Hoffbrand AV: Oxidative effect of iron on erythrocytes. Free Radic Res Commun 1985;1:55–62.
5.
Khalifa AS, El-Alfy MS, Moktar G, Fakeir AA, Khazbak MA, El-Baz F, El-Kholy M: Effect of desferrioxamine B on hemolysis in glucose-6-phosphate dehydrogenase deficiency. Acta Haematol 1989;82:113–116.
6.
Ekert H, Rawlinson H: Desferrioxamine and favism. N Engl J Med 1985;312:12.
7.
Meloni T, Forteone G, Gaetani GF: Desferrioxamine and favism. Br J Haematol 1986;63:394–395.
8.
Rowly DA, Halliwell B: Formation of hydroxyl radical from hydrogen peroxide and iron salts by superoxide and ascorbate dependent mechanism: Relevant to the pathology of rheumatoid disease. Clin Sci 1983;64:649–653.
9.
Novok T, Chevion M: Transition metals mediated enzymatic inactivation caused by favism-inducing agents. Biochem Biophys Res Commun 1984;122:297–303.
10.
Hoffbrand AV, Wonke B: Results of long-term subcutaneous desferrioxamine therapy. Baillières Clin Haematol 1989;2:345–362.
11.
Felsenfeld AJ, Rodriguez M, Coleman M, Ross D, Liach F: Desferrioxamine therapy in hemodialysis patients with aluminum-associated bone disease. Kidney Int 1989;35:1371–1378.
12.
Hyman CB, Gonick HC, Neufeld N, Agness CL: Mineral balance (iron, aluminum, copper, zinc) after high-dose intravenous Desferal in a child with hemoglobin Hammersmith and Turner’s syndrome. Am J Pediatr Hematol Oncol 1989;11:450–455.
13.
Breithaupt H, Hekers H, Pralle H, Guttmann W, Bleyl H, Graef V, Jundt G: High dosage desferrioxamine therapy in a female patient with acquired aplastic anaemia and transfusion siderosis. Blut 1986;52:211–219.
14.
Clark IA, Cowden WB, Hunt NH, Maxwell LE, Makie EJ: Activity of divicine in Plasmodium vinckis-infected mice has implications for treatment of favism and epidemiology of G6PD deficiency. Br J Haematol 1984;56:479–487.
1999
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.