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Chronic Non-Spherocytic Hemolytic Anemia (CNSHA) is a rare inherited blood disorder where red blood cells break down prematurely due to enzyme deficiencies. It is caused by severe glucose-6-phosphate dehydrogenase (G6PD) deficiency, which makes red blood cells fragile and unable to survive normally. The Glucose-6-Phosphate Dehydrogenase Quantitative test is the most important test for diagnosing CNSHA because it measures the exact level of G6PD enzyme activity in red blood cells.
CNSHA is caused by inherited enzyme deficiencies in red blood cells, most commonly severe glucose-6-phosphate dehydrogenase (G6PD) deficiency. The G6PD enzyme protects red blood cells from oxidative damage, and when it is severely deficient, red blood cells become fragile and break down prematurely in the bloodstream. Unlike other types of hemolytic anemia, the red blood cells maintain their normal disc shape but still die early, leading to chronic anemia and ongoing symptoms throughout life.
The Glucose-6-Phosphate Dehydrogenase Quantitative test is the most important test for CNSHA because it directly measures the amount of G6PD enzyme activity in your red blood cells. This test identifies severe enzyme deficiency that causes the chronic breakdown of red blood cells characteristic of CNSHA. The quantitative measurement is essential because it determines the severity of the deficiency and helps healthcare providers understand how significantly your red blood cells are affected. By measuring exact enzyme levels rather than just detecting presence or absence, this test provides the critical information needed to confirm a CNSHA diagnosis and guide treatment strategies.
You should get tested if you experience ongoing fatigue, pale skin, jaundice (yellowing of skin or eyes), dark urine, or an enlarged spleen without an obvious cause. Testing is especially important if you have a family history of anemia or blood disorders, if you come from Mediterranean, African, or Asian ancestry where G6PD deficiency is more common, or if you have chronic anemia that does not respond to typical treatments like iron supplementation. Early diagnosis helps prevent complications from chronic anemia and allows you to avoid triggers that might worsen red blood cell breakdown.
What this means
Your G-6-PD enzyme levels are below the normal range, indicating a deficiency. This means certain foods, medications, and infections can trigger your red blood cells to break down, causing anemia, fatigue, and other symptoms. Knowing this helps you avoid specific triggers and prevent future episodes.
Recommended actions
Avoid fava beans, certain legumes, and known trigger foods
Consult your doctor before taking new medications, especially antibiotics and antimalarials
Carry a list of medications and substances to avoid with you at all times
Inform family members about potential hereditary risk and consider genetic counseling
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