Adult Hemophagocytic Lymphohistiocytosis Mimicking Acute Viral Hepatitis.
Humans
Adult
Hepatitis, Viral, Human
Lymphohistiocytosis, Hemophagocytic
/ diagnosis
Ferritins
/ blood
Bone Marrow
/ pathology
Dexamethasone
/ therapeutic use
Anti-Inflammatory Agents
/ therapeutic use
Etoposide
/ therapeutic use
Diagnosis, Differential
Anorexia
/ etiology
Muscle Weakness
/ etiology
Weight Loss
Treatment Outcome
Journal
Digestive diseases and sciences
ISSN: 1573-2568
Titre abrégé: Dig Dis Sci
Pays: United States
ID NLM: 7902782
Informations de publication
Date de publication:
08 2023
08 2023
Historique:
received:
20
01
2023
accepted:
22
05
2023
medline:
19
7
2023
pubmed:
8
6
2023
entrez:
7
6
2023
Statut:
ppublish
Résumé
Adult hemophagocytic lymphohistiocytosis is a lifethreatenning disease which has hepatic manifestations mimicking acute hepatitis or can present with fulminant hepatic failure. The undelying pathophysiology is immune dysregulation causing a hyperinflammatory state. Clues to diagnosis include extremely high ferritin levels, whereas definitive diagnosis is usually made by bone marrow, as opposed to liver biopsy. Even with early and appropriate treatment with weekly dexamethasone and etoposide, mortality remains high.
Identifiants
pubmed: 37286930
doi: 10.1007/s10620-023-07985-3
pii: 10.1007/s10620-023-07985-3
doi:
Substances chimiques
Ferritins
9007-73-2
Dexamethasone
7S5I7G3JQL
Anti-Inflammatory Agents
0
Etoposide
6PLQ3CP4P3
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
3205-3207Informations de copyright
© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
Références
La Rosée P, Horne A, Hines M et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019;133:2465–2477. https://doi.org/10.1182/blood.2018894618 . (Epub 2019 Apr 16 PMID: 30992265).
doi: 10.1182/blood.2018894618
pubmed: 30992265
Ramos-Casals M, Brito-Zerón P, López-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet. 2014 Apr 26;383:1503–1516. doi: https://doi.org/10.1016/S0140-6736(13)61048-X . Epub 2013 Nov 27. Erratum in: Lancet. 2014 Apr 26;383:1464. PMID: 24290661.
Henter JI, Horne A, Aricó M et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124–131. https://doi.org/10.1002/pbc.21039 . (PMID: 16937360).
doi: 10.1002/pbc.21039
pubmed: 16937360