PB2039: INFLAMMATORY MARKERS IN HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS - A SINGLE CENTRE STUDY
Notice bibliographique
Résumé
Topic: 12. Bone marrow failure syndromes incl. PNH - Clinical Background: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathological immune activation that can be difficult to distinguish from other cytokine storm syndromes such as Adult-Onset Still’s disease (AOSD) and COVID-19 cytokine storm (CCS). The HLH-2004 criteria and HScore are the best available diagnostic criteria but have important limitations. Many of the tests included in the current HLH-2004 criteria, such as flow cytometry for NK cell cytotoxicity and cytokine analysis, are only available in specialized centers, and while useful in pediatric HLH, are less so in adult HLH which is characterized by hyperinflammation. Inflammatory markers such as ferritin, are not specific for HLH and can be seen in several hyperinflammatory syndromes. We sought to examine the clinical utility of a simply, readily available inflammatory marker, C-reactive protein CRP) in combination with sIL2r levels in distinguishing HLH from two similar cytokine storm syndromes, AOSD and CCS. Aims: Our aim is to analyze patterns of elevation in CRP and sIL2r to help clinicians in the differential diagnosis of HLH, AOSD, and CCS. Methods: A retrospective chart review was conducted for 61 patients with secondary HLH, 10 patients with AOSD, and 13 patients with CCS. Demographic data as well as inflammatory biomarkers including CRP and sIL2r levels were collected if drawn within 72 hours of the acute episode that led to diagnosis, and prior to treatment. The Kruskal-Wallis test was used to compare CRP in the HLH, AOSD, and CCS groups, as well as to compare CRP in HLH subgroups by underlying trigger (infection, malignancy, autoimmune, and idiopathic). The Kruskal-Wallis test was also used to measure and compare sIL2r levels in the HLH group compared to AOSD, as well as by HLH subtype. Results: C-reactive protein is significantly lower in secondary HLH (Mdn = 76.4) compared to AOSD (Mdn = 114.5, p = 0.039) and CCS (Mdn = 121.0, p = 0.003). When analyzed by subtype, CRP levels in malignancy-associated HLH (MAHS) were not significantly different than AOSD or CCS, while CRP levels were significantly lower in the non-MAHS groups (Mdn = 58.9, p = 0.01) compared to AOSD and CCS (p <0.001), particularly when compared to infection-associated HLH (IAHS) (Mdn = 32.7, p = 0.009). Soluble IL2 receptor levels were significantly higher in the HLH group (Mdn = 7436.0, p = 0.03) compared to AOSD (Mdn = 2252.0). Summary/Conclusion: C-reactive protein is significantly lower in HLH compared to AOSD and CCS, specifically in non-MAHS, and may be a useful and easily accessible biomarker aiding in the differential diagnosis of HLH. Soluble IL2 receptor levels are a useful biomarker in distinguishing AOSD from HLH, as it is not as significantly elevated in AOSD. Keywords: Macrophage, Inflammation, IL-6, Cytokine
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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) | 0,000 | 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) | 0,002 | 0,002 |
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; les deux têtes enseignantes s’accordent sur ce qui est montré ici.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».