The Safety of Chelators for Iron Overload in Sickle Cell Disease: A Brief Systematic Review

Özet

Sickle cell disease is a group of disorders that affects hemoglobin due to a mutation of the hemoglobin beta gene on chromosome 11. Patients have atypical hemoglobin molecules called hemoglobin S, which distort erythro-cytes into a “sickle-shape”. Typical symptoms of disease include periodic ep-isodes of pain, repeated infections, and anemia. This disorder is abundant in sub-Saharan African countries, the Mediterranean region, and also appears in some southern provinces in Turkey. Because of the high concentration of hemoglobin S in patients, a high risk of chronic anemia and vaso-occlusive events, such as stroke may deteriorate suddenly. In these conditions, trans-fusion of blood, especially erythrocytes, can be life-saving. However, chronic blood transfusions may lead to iron overload in patients. Erythrocyte trans-fusion is associated with a higher risk in most patients with sickle cell disease than in the general population. Therefore, chelation therapy has become an important component of the transfusion program to prevent complica-tions of iron accumulation in organs such as liver and heart. In this study, we sought to conduct a systematic review to assess the safety of iron chelat-ing agents used by patients with iron overload mainly due to necessary blood transfusion regime. Our evaluation revealed that in general iron chelation therapy, either deferasirox, deferoxamine or deferiprone, remains the most effective and safest available method to treat iron overload in sickle cell dis-ease. Furthermore, current reports do not reflect any significant safety con-cerns against the use of available chelators.