5607225 ADDITION OF THERAPEUTIC PLASMA EXCHANGE TO RED CELL EXCHANGE IMPROVES OUTCOMES OF FAT EMBOLISM SYNDROME IN SICKLE CELL DISEASE
Notice bibliographique
Résumé
Background: Fat embolism syndrome (FES) is a severe but possibly underdiagnosed complication of sickle cell disease (SCD) associated with very high mortality and morbidity. It is the result of extensive bone marrow necrosis that leads to release of large amounts of fat droplets in the systemic circulation. These not only cause mechanical obstruction of the microvasculature but also act as a substrate for the generation of various pro-inflammatory cytokines that contribute to the development of multiorgan failure. FES predominantly affects non-homozygous patients and those with a previously mild course of their illness. Immediate red cell exchange (RCE) can be life-saving and it is currently the treatment of choice. However, despite its increasing use in recent years, mortality remains substantial while a large number of survivors is left with significant neurocognitive impairment. With that in mind, we have previously advocated the addition of therapeutic plasma exchange (TPE) to RCE in order to address the inflammatory environment and potentially improve outcomes. Aims: To compare outcomes from FES using standard treatment with RCE and combination of RCE and TPE. Methods: A systematic review of all cases published since 2012 where RCE was employed was performed, and outcomes were compared to cases where a combination of RCE and TPE was used. Results: The systematic review of the literature identified 39 cases treated with RCE since 2012. Mortality was 21%, 33% of patients were left with severe and 13% with mild neurological impairment while only 33% made a complete recovery (CR). We are aware (publications/ personal communication) of 11 cases (separate from the previous 39) treated with combination of RCE and TPE worldwide (8 UK, 2 Canada, 1 USA). 9 patients were adults and 2 patients were children. 5 patients had homozygous Hb SS disease, 5 Hb SC and 1 Hb Sβ+. All patients, irrespective of genotype, had previously a mild course of their illness. The number of TPE procedures performed varied greatly between cases (1-10). There was 1 death (9%), 2 patients (18%) suffered mild/moderate neurological impairment and 8 patients (73%) achieved CR. Both patients reported as suffering mild/moderate neurological impairment are patients who developed the complication recently, have fully recovered cognition but are still in rehabilitation improving from generalised weakness resulting from prolonged hospitalisation and are very likely to achieve CR eventually. One more 13-year old patient (not included in this analysis as the final outcome is yet unknown) is currently recovering in hospital having recently suffered severe FES with multiorgan failure requiring extracorporeal membrane oxygenation now extubated, recovering organ function and able to communicate appropriately. Interestingly, 2 patients had a cytotoxic lesion of the corpus callosum (CLOCC) on brain imaging. This further highlights the role of the inflammatory environment in the pathophysiology of FES as CLOCCs are known to be the result of direct cytotoxicity from proinflammatory cytokines, especially IL-1 and IL-6. TPE was well tolerated with no associated adverse events. Conclusion: These early data suggest a clear benefit from this approach, and as TPE is a low risk intervention, we recommend its addition to RCE as standard management for FES in SCD. Reference 1. Tsitsikas DA, Vize J, Abukar J. Fat Embolism Syndrome in Sickle Cell Disease. J Clin Med. 2020 Nov 8;9(11):3601.
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Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,000 | 0,001 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,001 | 0,000 |
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 tête enseignante, 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 ».