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G6PD Deficiency

Background

Most common inherited enzyme deficiency affecting red blood cells. G6PD is a critical antioxidant—a deficiency can predispose to oxidation and subsequent hemolysis of the red blood cell.

Common oxidants include:
sulfonamides, furantoins, chloramphenicol, large doses of ascorbic acid, dapsone(>200mg/day), chloroquine, methylene blue, nalidixic acid, penicillamine, primaquine, quinadine & quinine.

The degree of hemolysis induced by a drug may be accentuated by the presence of additional factors (infection or disease state etc).The severity of the reaction is dependent on the type of G6PD deficiency (Mediterranean deficiency-Caucasian (most severe) ; Blacks (usually mild to moderate). The sex of the patient is also important—males are at greater risk based on severity compared to females.

Conclusion: G6PD is not an absolute contraindication to the use of oxidizing agents. Decisions should be based on a risk vs benefit analysis (consider severity of disease; sex of patient; availability of other agents; type of deficiency). If therapy is initiated, the patient should be monitored closely for adverse effects. Patients with G6PD deficiency will exhibit signs within 1-3 days of initiation of treatment. Symptoms may include abdominal or back pain in severe cases. The urine of the patient will darken in color.

References

1) Aderka D, Garfinkel D, Bograd H, Friedman J, Pinkhas J. Isosorbide dinitrate-induced hemolysis in G6PD-deficient subjects. Acta Haematol. 1983;69(1):63-4.

2) Beutler E. G6PD: population genetics and clinical manifestations. Blood Rev. 1996 Mar;10(1):45-52.

3) Eldad A, Neuman A, Weinberg A, Benmeir P, Rotem M, Wexler MR. Silver sulphadiazine-induced haemolytic anaemia in a glucose-6-phosphate dehydrogenase-deficient burn patient. Burns. 1991 Oct;17(5):430-2.

4) Grossman S, Budinsky R, Jollow D. Dapsone-induced hemolytic anemia: role of glucose-6-phosphate dehydrogenase in the hemolytic response of rat erythrocytes to N-hydroxydapsone. J Pharmacol Exp Ther. 1995 May;273(2):870-7.

5) Herman J, Ben-Meir S. Overt hemolysis in patients with glucose-6-phosphate dehydrogenase deficiency: a survey in general practice. Isr J Med Sci. 1975 Apr;11(4):340-6.

6) Hohl RJ, Kennedy EJ, Frischer H. Defenses against oxidation in human erythrocytes: role of glutathione reductase in the activation of glucose decarboxylation by hemolytic drugs. J Lab Clin Med. 1991 Apr;117(4):325-31.

7) Lavelle KJ, Atkinson KF, Kleit SA. Hyperlactatemia and hemolysis in G6PD deficiency after nitrofurantoin ingestion. Am J Med Sci. 1976 Sep-Oct;272(2):201-4.

8) Magon AM, Leipzig RM, Zannoni VG, Brewer GJ. Interactions of glucose-6-phosphate dehydrogenase deficiency with drug acetylation and hydroxylation reactions. J Lab Clin Med. 1981 Jun;97(6):764-70.

9) Myat-Phone-Kyaw, Myint-Oo, Aung-Naing, Aye-Lwin-Htwe. The use of primaquine in malaria infected patients with red cell glucose-6-phosphate dehydrogenase (G6PD) deficiency in Myanmar. Southeast Asian J Trop Med Public Health. 1994 Dec;25(4):710-3.

10) Reinke CM, Thomas JK, Graves AH. Apparent hemolysis in an AIDS patient receiving trimethoprim/sulfamethoxazole: case report and literature review. J Pharm Technol. 1996 Nov-Dec;11(6):256-62; quiz 293-5.

11) Tabbara IA. Related Articles Hemolytic anemias. Diagnosis and management. Med Clin North Am. 1992 May;76(3):649-68.

12) Vanella A, Campisi A, Castorina C, Sorrenti V, Attaguile G, Samperi P, Azzia N, Di Giacomo C, Schiliro G. Antioxidant enzymatic systems and oxidative stress in erythrocytes with G6PD deficiency: effect of deferoxamine. Pharmacol Res. 1991 Jul;24(1):25-31.
 

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David F. McAuley, Pharm.D., R.Ph.  GlobalRPh Inc.
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