Favism
Hello! Is G6PD deficiency considered a type of hemolytic anemia? Thank you.
kino.com, 20~29 year old female. Ask Date: 2003/03/07
Dr. Tang Jieru reply Cardiology
Hello: The correct medical term for "蠶豆症" is "Glucose-6-phosphate dehydrogenase (G6PD) deficiency." This condition is caused by a congenital deficiency of this enzyme, leading to metabolic dysfunction within red blood cells, making them susceptible to destruction by fava beans and certain medications, resulting in hemolysis.
It is classified as a type of hemolytic anemia.
Reply Date: 2003/03/07
More Info
Favism, also known as fava bean disease, is indeed a type of hemolytic anemia that is primarily triggered by the consumption of fava beans (Vicia faba) in individuals who have a deficiency in the enzyme glucose-6-phosphate dehydrogenase (G6PD). This condition is particularly prevalent in certain populations, especially those of Mediterranean, African, and Middle Eastern descent.
To understand favism and its relationship to hemolytic anemia, it's essential to delve into the underlying mechanisms. G6PD is an enzyme that plays a crucial role in the pentose phosphate pathway, which is vital for maintaining the integrity of red blood cells (RBCs). This pathway helps produce NADPH, which protects RBCs from oxidative damage. In individuals with G6PD deficiency, exposure to oxidative stress—such as that caused by certain foods (like fava beans), infections, or certain medications—can lead to the destruction of red blood cells, resulting in hemolytic anemia.
When a person with G6PD deficiency consumes fava beans, the compounds within these beans can induce oxidative stress, leading to the formation of reactive oxygen species (ROS). These ROS can damage the cell membranes of red blood cells, causing them to rupture (hemolyze). The symptoms of hemolytic anemia can vary in severity but often include fatigue, pallor, jaundice (yellowing of the skin and eyes), dark urine, and an increased heart rate. In severe cases, it can lead to acute hemolytic crises, which may require medical intervention.
Diagnosis of favism typically involves a combination of clinical history, laboratory tests, and genetic testing. Blood tests can reveal low hemoglobin levels, elevated bilirubin levels, and the presence of reticulocytes (immature red blood cells) as the body attempts to compensate for the loss of red blood cells. A specific test for G6PD activity can confirm the deficiency.
Management of favism primarily involves avoiding fava beans and other known triggers of oxidative stress. Patients are also advised to be cautious with certain medications that can exacerbate hemolysis, such as sulfa drugs and some antimalarials. In cases of acute hemolytic anemia, treatment may include supportive care, such as hydration and blood transfusions if necessary.
In summary, favism is a type of hemolytic anemia caused by G6PD deficiency, triggered by the consumption of fava beans. Understanding this condition is crucial for affected individuals to manage their health effectively and avoid potential complications. Regular follow-ups with healthcare providers and education about dietary restrictions and potential triggers can significantly improve the quality of life for those with this condition.
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