PB2045: USE OF ANAKINRA IN THE TREATMENT OF ADULT HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: A SYSTEMATIC REVIEW
Notice bibliographique
Résumé
Topic: 12. Bone marrow failure syndromes incl. PNH - Clinical Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome characterized by extreme inflammation and tissue destruction through immune activation of T cells, NK cells, and macrophages leading to a cytokine storm. HLH is traditionally classified as primary and secondary. While primary HLH is typically familial and seen in pediatric populations, secondary HLH is often caused by autoimmune diseases, infectious diseases, and malignancies. While underlying incidence is very low, HLH is associated with significant mortality reaching near 100% when untreated, and 50-75% with traditional chemotherapy-based treatment regimens. Recently, treatment regimens have increasingly included Anakinra, an IL-1 Receptor antagonist. The evidence for the use of Anakinra is primarily founded upon pediatric literature, while there is very limited evidence for its use in adult patients. Aims: To synthesize the existing primary evidence for Anakinra use in adult patients with HLH. Methods: A comprehensive search of MEDLINE, Embase, and the grey literature was done from inception to December 2022. Studies were screened at abstract and full-text level independently and in duplicate. All English language retrospective cohort, prospective cohort, and randomized controlled studies investigating the use of Anakinra in adult patients with HLH and related hemophagocytic disorders (I.e., macrophage activation syndrome) were included. Baseline characteristics, underlying diagnoses, HLH lab parameters, details of Anakinra regimen, concurrent therapy, and comparator group therapy were recorded. The primary outcome measure was mortality as defined by the primary study. Secondary outcomes include length of hospitalization, length of ICU stay, clinical remission, relapse rate, and resolution of inflammatory markers. Results: Of the 1591 studies screened, eight studies with a total of 81 unique patients were included in the analysis. Studies included patients with various etiologies of HLH, most commonly autoimmune, malignant, and infectious. The median Anakinra dosage was 530 mg total per day (range: 100mg, 2400 mg) given either subcutaneously, intravenously, or via intravenous infusion. Steroids were included as concurrent therapy in all studies, with some studies including other immunosuppressive therapy including IVIG, cyclosporine, cyclophosphamide, tocilizumab, etoposide, and ruxolitinib. Median mortality rate was 45% (Range: 12.5%, 69%). Total mortality rate of all included patients was 42% (34/81). Summary/Conclusion: Regardless of available treatment HLH is associated with very high mortality. Given the low incidence of HLH and importance of individual patient factors, treatment regimens need to be personalized. Anakinra may have benefit in conjunction with conventional therapy in certain cases. Given its relative ease of administration and low toxicity as compared to chemotherapy-based regimens, use of Anakinra may be preferred. Evidence regarding potential benefit and dosing regimens is extremely limited. Further observational studies are required to reliably demonstrate the efficacy of Anakinra in HLH.Keywords: Immunosuppressive therapy, Hemophagocytic Lymphohistiocytosis (HLH)
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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 | 0,001 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,006 | 0,001 |
| 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) | 0,000 | 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 ».