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Sickle cell disease (SCD) is an inherited blood disorder that affects ~100,000 Americans (1, 2). In SCD, red blood cells (RBC) containing aberrant sickle hemoglobin (HgbS) become sickle-shaped at low oxygen tension and stick together leading to obstructed blood flow. The resulting decrease in tissue oxygenation causes chronic complications such as vasculo-occlusive pain crises among others that require frequent hospitalizations (3). Chronic RBC transfusions play a prominent role in the treatment of this disease by improving oxygenation through addition of normal RBC and dilution of RBC containing HgbS (4, 5). However, the major disadvantage of management with simple transfusion is the introduction of excess iron into the body which outpaces dedicated mechanisms for iron removal. Thus, over the course of the disease, excess iron is deposited into multiple organs causing organ damage leading to eventual organ failure. By contrast, automated red cell exchange (RCE) is an alternative therapeutic approach that exchanges the patient's sickle RBC with normal RBC reducing the sickle cell RBC more efficiently while being iron neutral (6). Despite the apparent advantage of RCE (7) and inclusion in current management recommendations (8), its benefits for patients with iron overload remains controversial. In this study we examined if RCE improved long-term survival for SCD patients with iron overload treated at our institution.
iron overload, mortality, red cell exchange, sickle cell, survival
Frontiers in Medicine
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Zhou YY, Reeves HM, Webb L, Santiago Z, and Maitta RW (2022) Chronic red cell exchange in sickle cell patients with iron overload may not affect mortality. Front. Med. 9. doi: 10.3389/fmed.2022.892967