Review of Therapeutic Erythrocytapheresis in Patients with Thalassemia Major
Notice bibliographique
Résumé
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.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction machine sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Le volet Gemma est une étiquette directe du modèle pour chaque travail de la base, lue sur la notice réduite au titre. Le volet Codex est un classifieur appris des 10 348 étiquettes directes de Codex et calibré sur les taux pondérés de l'échantillon; les champs sans appui suffisant ne portent aucun appel Codex. Le mode candidate est l'union des deux volets; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont pas des étiquettes humaines.
Scores du classifieur distillé par catégorie (deux têtes)
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,003 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,001 |
| Bibliométrie | 0,004 | 0,005 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,001 | 0,001 |
| Science ouverte | 0,001 | 0,000 |
| Intégrité de la recherche | 0,001 | 0,001 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,002 | 0,001 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule source (Gemma direct ou Codex distillé), pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».