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D64.2 – Secondary Sideroblastic Anaemia
D64.2 is the ICD-10 code for secondary sideroblastic anaemia, a blood disorder in which iron cannot be properly incorporated into red blood cells and accumulates in bone marrow precursor cells.
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D64.2 is the ICD-10 code for secondary sideroblastic anaemia, a blood disorder in which iron cannot be properly incorporated into red blood cells and accumulates in bone marrow precursor cells.
What is D64.2 – Secondary Sideroblastic Anaemia?
The ICD-10 code D64.2 refers to secondary sideroblastic anaemia, a form of anaemia (low red blood cell count) in which red blood cells cannot be produced adequately. The underlying problem lies in the inability to incorporate iron correctly into haemoglobin, the oxygen-carrying protein in red blood cells. Instead, iron accumulates in a ring around the nucleus of red blood cell precursors (erythroblasts) in the bone marrow. These abnormal cells are called ring sideroblasts and are the hallmark of this condition. Unlike hereditary (congenital) forms, secondary sideroblastic anaemia is caused by an external factor such as a medication, toxin, or underlying disease.
Causes
Secondary sideroblastic anaemia is triggered by factors that interfere with iron metabolism or haemoglobin synthesis in the bone marrow. Common causes include:
- Medications and substances: Certain antibiotics (e.g., chloramphenicol), tuberculosis drugs (e.g., isoniazid), alcohol, and some chemotherapy agents can disrupt red blood cell maturation.
- Lead poisoning: Lead inhibits several enzymes essential for haem synthesis.
- Vitamin B6 deficiency (pyridoxine deficiency): Vitamin B6 is a key cofactor in haemoglobin synthesis.
- Copper deficiency: Low copper levels can impair iron utilisation in the bone marrow.
- Chronic diseases: Rheumatoid arthritis, myelodysplastic syndromes, and inflammatory bowel diseases may be associated.
- Chronic alcohol use: One of the most common causes of secondary sideroblastic anaemia.
Symptoms
The symptoms of secondary sideroblastic anaemia resemble those of other types of anaemia and vary in severity:
- Fatigue and increased exhaustion
- Pallor of the skin and mucous membranes
- Shortness of breath on exertion
- Rapid heartbeat (palpitations)
- Dizziness and headaches
- Over time, organ damage due to iron accumulation (haemosiderosis) in the liver, heart, or pancreas
Diagnosis
Diagnosis is made through a combination of laboratory tests and bone marrow examination:
- Blood count: Anaemia is detected, often with normal or elevated serum iron markers (elevated ferritin and transferrin saturation).
- Bone marrow biopsy: Ring sideroblasts are identified using Prussian blue (Berlin blue) staining – more than 15% ring sideroblasts are diagnostically significant.
- Cause investigation: Medication history, blood lead and copper levels, vitamin B6 levels, and alcohol history are assessed.
Treatment
Treatment of secondary sideroblastic anaemia is directed primarily at the underlying cause:
- Discontinuing the causative medication: Stopping the responsible drug often leads to significant improvement.
- Abstaining from alcohol: For alcohol-related anaemia, abstinence is the most important intervention.
- Vitamin B6 supplementation: Effective when pyridoxine deficiency is confirmed or when the condition is caused by isoniazid therapy.
- Treating the underlying disease: Managing associated conditions such as rheumatoid arthritis or inflammatory bowel disease.
- Chelation therapy: Iron-chelating agents may be used in cases of severe iron overload.
- Blood transfusions: Red blood cell transfusions may be required in cases of severe anaemia.
Prognosis
The prognosis of secondary sideroblastic anaemia is generally favourable when the underlying cause is identified and treated promptly. Once the triggering factor is removed, normal blood cell production often resumes. Regular medical follow-up is important to monitor for potential organ iron overload and to ensure full recovery.
References
- World Health Organization (WHO): ICD-10 Version 2019 – D64.2 Secondary sideroblastic anaemia. Available at: https://icd.who.int/browse10/2019/en#/D64.2
- Hoffbrand AV, Moss PAH: Hoffbrand's Essential Haematology, 8th Edition. Wiley-Blackwell, 2020.
- Bottomley SS, Fleming MD: Sideroblastic anemias. In: Hematology: Basic Principles and Practice, 7th Edition. Elsevier, 2018.
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