Review of Therapeutic Erythrocytapheresis in Patients with Thalassemia Major
Bibliographic record
Abstract
Introduction Therapeutic erythrocytapheresis, or red cell exchange (RCE) is a non-invasive procedure in which a patient's erythrocytes are removed from the bloodstream while being replaced with erythrocytes from blood donors. RCE is commonly used as a transfusion technique in patients with sickle cell disease (SCD) to help treat and prevent complications associated with sickling of erythrocytes and iron overload. In the rare blood disorders (RBD) clinic at the University of Alberta in Edmonton, Canada, RCE has been utilized as a therapeutic adjunct to traditional chelation therapies for patients with thalassemia major with uncontrolled iron overload. The aim of this study is to describe and review the rate of observed complications or benefits associated with RCE in patients with thalassemia major. Methods Patients with a documented diagnosis of thalassemia major who were enrolled in the RCE program via the RBD clinic were identified. Charts were retrospectively reviewed from time of initiation of RCE to the time of most recent RCE or clinic follow up. Complications were documented and analyzed, then compared to standard rates of complications of iron overload in patients with thalassemia major. Results Seven (7) patients with were identified for analysis. The average age of patients enrolled was 30 years. One hundred percent (100%) of patients had confirmed diagnosis of thalassemia major by hemoglobin electrophoresis. 85.7% of patients were of male sex. Six (85.7%) patients were on chelation therapy (3 patients on Jadenu plus Ferriprox; 3 patients on Jadenu alone). On average RCE was performed every 3.85 weeks. Of the patients analyzed during RCE, 85.7% had hepatic iron overload and 42.9% had cardiac dysfunction. 71.4% had evidence of extramedullary hematopoiesis. Two patients had syncopal events while on RCE. Two patients developed red cell antibodies during RCE. Improvements in iron overload correlated with initiation of dual chelation therapy in the three patients who were on maximal dual chelation. Conclusions Although RCE is a useful transfusion strategy in patients with SCD with less iron loading than simple transfusion, it does not appear to have the same benefits in patients with thalassemia major. Improvements in myocardial and hepatic T2* scores correlated with being on dual chelation therapy rather than with the frequency or duration of RCE. There was evidence of significant extramedullary hematopoiesis in 5 of the 7 patients on RCE possibly due to poor hemoglobin increment. Given the observed increase in extramedullary hematopoiesis, syncope, and antibody formation associated with RCE, and lack of benefit in reducing iron overload, we do not recommend its use as a treatment in patients with thalassemia major. Disclosures No relevant conflicts of interest to declare.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.004 | 0.005 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.002 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".