Guidelines for Iron Therapy in Alpha Thalassemia
Iron therapy in alpha thalassemia should be avoided unless there is documented iron deficiency, as these patients are at risk for iron overload despite having anemia. 1, 2
Assessment of Iron Status in Alpha Thalassemia
- Patients with alpha thalassemia may have misleading serum ferritin levels that don't accurately reflect their true iron burden, necessitating more advanced testing 3
- MRI-based liver iron concentration (LIC) measurement is the preferred method for assessing iron status in alpha thalassemia patients, as serum ferritin correlations are modest and vary by genotype 3
- Cardiovascular magnetic resonance (CMR) imaging with T2* technique should be used to assess cardiac iron overload, with T2* <20 milliseconds indicating increased risk of decreased left ventricular ejection fraction 4
Iron Therapy Guidelines for Alpha Thalassemia
When Iron Therapy IS Indicated:
- Iron supplementation should ONLY be given when there is documented iron deficiency (not just anemia) in alpha thalassemia patients 1, 2
- If iron deficiency is confirmed, oral ferrous sulfate at lower doses than standard (e.g., 200 mg once daily rather than twice daily) may be better tolerated while still effective 4
- Iron therapy should be continued only until iron deficiency is corrected, then discontinued to prevent iron overload 4
When Iron Therapy is CONTRAINDICATED:
- Patients with alpha thalassemia without documented iron deficiency should NOT receive iron supplementation, as they are at risk for iron overload despite their anemia 1, 2
- Vitamin C supplements should be avoided in alpha thalassemia patients at risk for iron overload, as they can enhance iron absorption 4
Monitoring During Iron Therapy
- Regular monitoring of serum ferritin every 3 months during iron therapy 1
- MRI assessment of liver iron concentration should be performed before starting iron therapy and periodically thereafter 3
- Cardiac T2* MRI should be performed annually in transfusion-dependent patients 1
Iron Chelation in Alpha Thalassemia
- Iron chelation therapy should be initiated in transfusion-dependent alpha thalassemia patients who have received 20-60 RBC concentrates or if serum ferritin rises above 1000-2500 μg/L 4
- Oral chelators (particularly deferasirox) are preferred for most patients due to better compliance and effectiveness 5, 6
- Deferoxamine may be preferred during periods of acute illness or in patients with renal dysfunction 4
- Deferiprone should be used with caution due to risk of neutropenia 1, 2
Special Considerations
- Non-deletional alpha thalassemia genotypes may have higher liver iron concentrations compared to deletional forms at the same serum ferritin levels, requiring different thresholds for initiating chelation therapy 3
- Patients with alpha thalassemia who are candidates for stem cell transplantation should be chelated early to prevent transplant-related mortality associated with iron overload 4
- Cardiac complications from iron overload are a major cause of death in transfusion-dependent thalassemia patients, making cardiac monitoring essential 2, 7
Common Pitfalls
- Assuming that anemia in alpha thalassemia indicates iron deficiency - this is a dangerous misconception that can lead to inappropriate iron supplementation 1, 2
- Relying solely on serum ferritin to guide iron therapy decisions in alpha thalassemia - MRI assessment provides more accurate information 3
- Failing to initiate iron chelation therapy concurrently with transfusion therapy in transfusion-dependent patients 2, 8
- Inadequate monitoring of iron burden during chelation therapy, which can lead to either insufficient chelation or chelator-related toxicity 8