Diagnostic Challenges in Pediatric Hemophagocytic Lymphohistiocytosis.
Ferritin
hemophagocytic lymphohistiocytosis (HLH)
immune dysregulation [3–6]
Journal
Journal of clinical immunology
ISSN: 1573-2592
Titre abrégé: J Clin Immunol
Pays: Netherlands
ID NLM: 8102137
Informations de publication
Date de publication:
08 2021
08 2021
Historique:
received:
16
11
2020
accepted:
15
03
2021
pubmed:
25
3
2021
medline:
4
2
2022
entrez:
24
3
2021
Statut:
ppublish
Résumé
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune dysregulation that encompasses a broad range of underlying genetic diseases and infectious triggers. Monogenic conditions, autoimmune diseases, and infections can all drive the phenotype of HLH and associated immune hyperactivation with hypercytokinemia. A diagnosis of HLH usually requires a combination of clinical and laboratory findings; there is no single sensitive and specific diagnostic test, which often leads to "diagnostic dilemmas" and delays in treatment initiation. Ferritin levels, one of the most commonly used screening tests, were collected across a large tertiary care pediatric hospital to identify the positive predictive value for HLH. Herein, we present several cases that illustrate the clinical challenges of confirming an HLH diagnosis. Additionally, we report on the utility of establishing a formal multi-disciplinary group to aid the prompt diagnosis and treatment of patients presenting with HLH-like pathophysiologies.
Identifiants
pubmed: 33761058
doi: 10.1007/s10875-021-01025-3
pii: 10.1007/s10875-021-01025-3
pmc: PMC7988244
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, Non-P.H.S.
Langues
eng
Sous-ensembles de citation
IM
Pagination
1213-1218Subventions
Organisme : NCI NIH HHS
ID : R01 CA193776
Pays : United States
Organisme : NCI NIH HHS
ID : U01 CA232486
Pays : United States
Informations de copyright
© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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