D55.1
Anemia due to other disorders of glutathione metabolism
Clinical Classification Guidelines
Inclusion Terms
- Anemia (due to) enzyme deficiencies, except G6PD, related to the hexose monophosphate [HMP] shunt pathway
- Anemia (due to) hemolytic nonspherocytic (hereditary), type I
Medical Intelligence & Overview
Anemia due to other disorders of glutathione metabolism, classified under ICD-10 code D55.1, is a rare condition where a person's body does not produce enough red blood cells because of issues related to glutathione processing. Glutathione is an important antioxidant that helps protect cells from damage. When this process is disrupted, it can lead to anemia, a condition characterized by a lack of healthy red blood cells necessary for transporting oxygen throughout the body. This type of anemia is linked to enzyme deficiencies related to the hexose monophosphate (HMP) shunt pathway, excluding G6PD deficiency, and may also be associated with hereditary hemolytic conditions causing red blood cell destruction.
Causes & Symptoms
Clinical Causes: Enzyme deficiencies affecting glutathione metabolism, specifically outside of G6PD deficiency Disorders of the hexose monophosphate (HMP) shunt pathway Hereditary hemolytic anemia not caused by spherocytes Genetic mutations impacting enzymes involved in glutathione processing
Key Symptoms: Fatigue and weakness from decreased oxygen delivery Paleness or pallor of the skin and mucous membranes Shortness of breath, especially during exertion Rapid heart rate (tachycardia) Jaundice, indicated by yellowing of the skin and eyes Dark-colored urine due to hemoglobin breakdown products Enlarged spleen (splenomegaly) in some cases Episodes of hemolytic crisis or sudden increase in red blood cell destruction
Diagnostic & Treatment
Diagnosis Path: Diagnosing anemia caused by disorders of glutathione metabolism involves blood tests to measure levels of red blood cells and hemoglobin. Additional tests may include:
Treatment Protocols: Management focuses on controlling hemolysis and alleviating symptoms. Treatment options may include:
Clinical Advice & FAQs
Billing Guidance
Is D55.1 a billable ICD-10 code?
Yes, D55.1 is a specific, billable code that can be used to indicate a diagnosis for reimbursement purposes.
Documentation
How do I report D55.1?
Clinical documentation must specify the nature of Anemia due to other disorders of glutathione metabolism and any associated comorbidities for accurate reporting.
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