Approach Considerations

Most individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency do not need treatment. However, they should be taught to avoid drugs and chemicals that can cause oxidant stress. Patients should also avoid broad beans (ie, fava beans). Favism occurs primarily in the Mediterranean variety of G6PD deficiency.

Identification and discontinuation of the precipitating agent is critical to manage hemolysis in patients with G6PD deficiency. Anemia should be treated with appropriate measures, recognizing that hemolysis is self-limited and often resolves in 8 to 14 days. Transfusions are rarely indicated. Splenectomy is usually ineffective.

Infants with prolonged neonatal jaundice as a result of G6PD deficiency should receive phototherapy with a bili light (see Neonatal Jaundice). Exchange transfusion may be necessary in cases of severe neonatal jaundice or hemolytic anemia caused by favism. However, Samanta and colleagues found that in neonates with idiopathic hyperbilirubinemia, transfusion with G6PD-deficient blood was significantly less effective than transfusion with G6PD-normal blood.^[32]

Patients with chronic hemolysis or non-spherocytic anemia should be placed on daily folic acid supplements. Consultations with a hematologist and a geneticist should be sought.

Deterrence/Prevention

Persons with G6PD deficiency need to avoid foods, drugs, and chemicals that can precipitate hemolysis. The risk posed by those substances is determined in part by the person's G6PD variant (see Etiology) and thus the degree of enzyme deficiency.

Fava beans are the food best known for precipitating hemolysis; indeed, favism—the term for symptomatic attacks of hemolytic anemia from eating fava beans—has been recognized since antiquity.^[10]Other foods that some persons with G6PD deficiency may prefer to avoid include the following^[33]:

Red wine
All legumes
Blueberries
Soya products
Tonic water

For most persons with G6PD deficiency, the following drugs pose a definite risk:^[1]

Dapsone and other sulfones (higher doses for dermatitis herpetiformis more likely to cause problems)
Methylthioninium chloride
Niridazole
Nitrofurantoin
Pamaquin
Primaquine (30 mg weekly for 8 weeks has been found to be without undue harmful effects in African and Asian people)
Quinolones (ciprofloxacin, moxifloxacin, nalidixic acid, norfloxacin, ofloxacin)
Rasburicase
Sulfonamides (including co-trimoxazole; however, some sulfonamides, such as sulfadiazine, have been found not to be hemolytic in many G6PD-deficient individuals)

Drugs that pose a possible risk in some persons with G6PD deficiency include the following:

Aspirin (acceptable up to a dose of at least 1g daily in most G6PD-deficient individuals)
Chloroquine (acceptable in acute malaria and malaria chemoprophylaxis)
Menadione (vitamin K3) and water-soluble derivatives (eg, menadol sodium phosphate)
Quinidine (acceptable in acute malaria)
Quinine (acceptable in acute malaria)
Sulfonylureas
Naphthalene in (mothballs)

Intravenous vitamin C is currently undergoing testing as adjunctive treatment for sepsis. Case reports describe episodes of hemolysis in patients with G6PD deficiency who received high doses of intravenous (IV) vitamin C (> 60 g). However, Marik cites dramatic reductions of methemoglobinemia and hemolysis that have been reported in patients treated with IV vitamin C in low to moderate doses (1-10 g every 6 hours), and proposes that those data suggest that vitamin C at a dosage of 6 g/day should not be considered contraindicated in patients with known or suspected G6PD deficiency—that indeed, IV vitamin C may be the treatment of choice in G6PD-deficient patients with drug-induced hemolysis. Sepsis can cause methemoglobinemia, and methylene blue—the first-line treatment for acquired methemoglobinemia—is contraindicated in patients with G6PD deficiency.^[34]

Medication

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Media Gallery

G6PD deficiency: Heinz bodies in a peripheral smear stained with a supravital stain. Heinz bodies are denatured hemoglobin, which occurs in G6PD deficiencies and in unstable hemoglobin disorders.

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Author

Srikanth Nagalla, MD, MS, FACP Chief of Benign Hematology, Miami Cancer Institute, Baptist Health South Florida; Clinical Professor of Medicine, Florida International University, Herbert Wertheim College of Medicine

Srikanth Nagalla, MD, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Alexion; Alnylam; Kedrion; Sanofi; Dova; Apellis; Pharmacosmos<br/>Serve(d) as a speaker or a member of a speakers bureau for: Sobi; Sanofi; Rigel.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, SWOG

Disclosure: Partner received none from No financial interests for none.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Karen Seiter, MD Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, American Society of Hematology

Disclosure: Received honoraria from Novartis for speaking and teaching; Received consulting fee from Novartis for speaking and teaching; Received honoraria from Celgene for speaking and teaching.

Paul Schick, MD † Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

Acknowledgements

Suzanne M Carter, MS Senior Genetic Counselor, Associate, Department of Obstetrics and Gynecology, Division of Reproductive Genetics, Montefiore Medical Center, Albert Einstein College of Medicine

Suzanne M Carter, MS is a member of the following medical societies: American Bar Association

Disclosure: Nothing to disclose. Susan J Gross, MD, FRCS(C), FACOG, FACMG Codirector, Division of Reproduction Genetics, Associate Professor, Department of Obstetrics and Gynecology, Albert Einstein College of Medicine

Susan J Gross, MD, FRCS(C), FACOG, FACMG is a member of the following medical societies: American College of Medical Genetics, American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, American Medical Association, American Society of Human Genetics, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Sections Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency
Overview
Presentation
DDx
Workup
Treatment
Medication
Questions & Answers
References

Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency Treatment & Management

Approach Considerations

Deterrence/Prevention

References

Tables

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