Complications of red cell exchange for anemia management in patients with transfusion-dependent thalassemia

In the Rare Blood Disorders clinic at the University of Alberta in Edmonton, red cell exchange (RCE) was utilized in transfusion-dependent thalassemia (TDT) patients with severe iron overload despite oral chelation and no access to iron infusion pumps for parenteral chelation. It was hypothesized that RCE would be less iron loading compared to simple transfusion. The purpose of this study is to document observations of the potential risks and benefits of RCE in TDT patients.

TDT patients treated with RCE were identified and consented for enrolment according to local research ethics standards. Seven patients were enrolled in the study. Charts were retrospectively reviewed from the time of initiation of RCE to the time of the most recent RCE or clinic follow-up. Outcomes were documented and analyzed by descriptive analysis.

The average age was 30 years. 85.7% were male. 100% were on oral chelation therapy and had hyperferritinemia at baseline. Outcomes included hepatic iron overload (5 of 7), cardiac dysfunction (3 of 7), worsening splenomegaly or extramedullary hematopoiesis (5 of 7), syncopal events during RCE (2 of 7), and new antibodies (1 of 7). Iron overload improved after escalated oral chelation, not in relation to RCE initiation.

We hypothesize complications were higher than expected due to inadequate hematocrit increment and lack of suppression of ineffective erythropoiesis. With no observed benefit in iron status, and high complication rates, we did not find evidence to recommend RCE in patients with TDT. This case series is a hypothesis-generating study on transfusion techniques in TDT.