Iron Therapy in Alpha Thalassemia
Iron supplementation should NOT be prescribed for alpha thalassemia patients unless there is documented iron deficiency, as these patients are at risk for iron overload despite having anemia. 1
Assessment Before Considering Iron Therapy
- Alpha thalassemia patients often have anemia that is mistakenly treated with iron, which can lead to dangerous iron overload 1
- Before considering iron therapy, confirm iron deficiency through laboratory testing (serum ferritin, transferrin saturation) rather than assuming deficiency based on anemia alone 1, 2
- In a study of thalassemia minors, 31% had coexisting iron deficiency, but 69% did not require iron supplementation 2
- Consider iron therapy only if ferritin is low (<30 ng/mL) and transferrin saturation is reduced (<16%) 1
Appropriate Iron Therapy When Deficiency is Confirmed
- If iron deficiency is documented in alpha thalassemia:
Monitoring for Iron Overload
- Regular monitoring of serum ferritin levels and number of red blood cell transfusions received is essential to assess iron overload 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 1, 3
- Annual cardiac T2* MRI is recommended in transfusion-dependent patients 1
Iron Chelation in Transfusion-Dependent Patients
- 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 1, 3
- Available chelation options include:
Special Considerations
- Patients who are candidates for stem cell transplantation should be chelated early to prevent transplant-related mortality associated with iron overload 3, 1
- Cardiac complications from iron overload are a major cause of death in transfusion-dependent thalassemia patients, making cardiac monitoring essential 3
- Combination therapy with deferoxamine and deferiprone has shown greater improvement in left ventricular ejection fraction than deferoxamine alone in beta-thalassemia patients with cardiac iron overload 5
Common Pitfalls to Avoid
- Assuming that anemia in alpha thalassemia indicates iron deficiency is a dangerous misconception 1, 2
- A practical screening approach: If Hb < 11.5 g/dL in a case of thalassemia minor, screen for iron deficiency (sensitivity 79.8%, specificity 82.6%) 2
- Failing to initiate iron chelation therapy concurrently with transfusion therapy in transfusion-dependent patients 1
- Using deferasirox in patients with renal failure 3