What it is
G6PD deficiency is an inherited difference in red blood cells in which oxidative stressors, such as certain drugs or eating fava beans, can trigger sudden episodes of red-cell breakdown (acute hemolysis).
Signs and symptoms
Hemoglobinuria
Hemoglobinuria means the urine turns dark because pigment from broken-down red blood cells passes into it. It can appear during an episode of red-cell breakdown.
Jaundice
Jaundice is a yellowing of the skin and the whites of the eyes that happens when red blood cells break down. It is one of the common features of G6PD deficiency, including in newborns.
Abdominal pain
Belly (abdominal) pain can occur during an acute hemolytic episode, and is more common in children whose episode was set off by fava beans.
Fava bean-induced hemolytic anemia
Favism is the name for an acute episode of red-cell breakdown set off by eating fava beans. It is a well-known cause of sudden hemolytic anemia in people with G6PD deficiency.
Treatment and management
No disease-modifying treatment is established for this condition in the research mapped here. This is a stated, reviewed fact, not a missing piece of this guide.
That does not mean nothing can be done. Supportive and symptomatic care, managing specific symptoms and complications as they arise, can still matter a great deal. What is right for any individual is a conversation for their own care team.
Triggers to avoid
In this condition, certain drugs, foods, or other exposures can set off an acute episode in people who are affected, even when they are otherwise well. The research mapped here describes the agents below. This is information, not a recommendation: what to avoid and what is safe for any individual is a conversation for their own care team.
Fava beans
Eating fava beans is a well-recognized trigger of acute hemolysis in people with G6PD deficiency and is something to avoid.
Reported in the research mapped here as able to provoke: Hemolytic anemia, G6PD deficient (favism) in people with this condition.
“…exposure to oxidative stressors, such as drugs or fava bean ingestion, can trigger acute hemolytic episodes…”
Methylene blue
Methylene blue is a drug that should be avoided in G6PD deficiency because it can worsen red-cell breakdown.
Reported in the research mapped here as able to provoke: Hemolytic anemia, G6PD deficient (favism) in people with this condition.
“These patients should not receive methylene blue to avoid worsening hemolysis.”
Dapsone
Dapsone is a drug used for some infections and immune conditions. It can trigger red-cell breakdown in people with G6PD deficiency and is one to avoid or use only with caution.
Reported in the research mapped here as able to provoke: Hemolytic anemia, G6PD deficient (favism) in people with this condition.
“Dapsone may cause haemolysis, particularly in individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency.”
Pegloticase
Pegloticase is a drug for severe gout. It can trigger oxidative red-cell breakdown in people with G6PD deficiency, so G6PD status should be checked before it is used.
Reported in the research mapped here as able to provoke: Hemolytic anemia, G6PD deficient (favism) in people with this condition.
“Pegloticase is an effective therapy for refractory gout but carries a risk of oxidative hemolysis in patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency.”
Turn this into questions for your doctor
The hardest part is often knowing what to ask. PatientLead Health helps families turn what is on this page into the right questions for their care team.
How to read the evidence labels
Where this comes from
This guide is built from 5 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.