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D55.0 – Anaemia due to G6PD Deficiency
D55.0 is the ICD-10 code for anaemia due to glucose-6-phosphate dehydrogenase (G6PD) deficiency, a hereditary condition causing premature destruction of red blood cells.
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D55.0 is the ICD-10 code for anaemia due to glucose-6-phosphate dehydrogenase (G6PD) deficiency, a hereditary condition causing premature destruction of red blood cells.
What is D55.0?
The ICD-10 code D55.0 refers to anaemia due to glucose-6-phosphate dehydrogenase (G6PD) deficiency. This is an inherited haemolytic anaemia caused by a defect in the enzyme G6PD, which plays a critical role in protecting red blood cells (erythrocytes) from oxidative damage. Without sufficient G6PD activity, red blood cells are prematurely destroyed.
Causes
D55.0 is caused by a deficiency or reduced activity of the enzyme glucose-6-phosphate dehydrogenase. The defect is inherited in an X-linked recessive pattern, meaning it predominantly affects males. The condition is most prevalent in tropical and subtropical regions, including sub-Saharan Africa, the Mediterranean, the Middle East, and South and Southeast Asia.
Common triggers for a haemolytic crisis include:
- Certain medications (e.g., primaquine, nitrofurantoin, some sulfonamides)
- Infectious diseases
- Consumption of fava beans – a reaction known as favism
- Exposure to oxidative chemicals
- Neonatal jaundice as an early manifestation in newborns
Symptoms
Many individuals with G6PD deficiency remain asymptomatic until exposed to a trigger. Acute haemolytic episodes can present with:
- Jaundice (icterus) due to elevated bilirubin levels
- Pale or yellowish skin and mucous membranes
- Dark urine (brown or red) caused by haemoglobin released from destroyed red blood cells
- Fatigue, weakness, and dizziness
- Shortness of breath and rapid heartbeat
- Abdominal or back pain in severe cases
Diagnosis
Diagnosis of D55.0 is established through:
- Full blood count: Evidence of anaemia with signs of haemolysis (elevated bilirubin, elevated LDH, reduced haptoglobin)
- Blood smear: Detection of characteristic Heinz bodies within red blood cells
- G6PD activity assay: Direct measurement of reduced enzyme activity in the blood
- Genetic testing: Confirmation of the underlying gene mutation
Treatment
There is currently no curative treatment for G6PD deficiency. Management focuses on preventing and managing haemolytic crises:
- Avoiding triggers: Strict avoidance of causative drugs, fava beans, and oxidative substances
- Supportive care: Adequate hydration and treatment of underlying infections
- Blood transfusions: Required in severe, life-threatening haemolytic episodes
- Folic acid supplementation: In chronic haemolytic anaemia to support red blood cell production
- Phototherapy: For newborns presenting with G6PD-related jaundice
Patients with known G6PD deficiency are advised to carry a medical alert card listing their diagnosis to prevent administration of incompatible medications in emergency situations.
References
- World Health Organization (WHO): Glucose-6-Phosphate Dehydrogenase Deficiency. WHO Working Group, Bulletin of the WHO, 1989.
- Luzzatto L, Nannelli C, Notaro R: Glucose-6-Phosphate Dehydrogenase Deficiency. Hematology/Oncology Clinics of North America, 2016.
- ICD-10-CM Official Guidelines for Coding and Reporting, FY2024. National Center for Health Statistics (NCHS), D55.0.
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