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Induction of Drug-Induced, Autoimmune Hepatitis in BALB/c Mice for the Study of Its Pathogenic Mechanisms
Published on: May 29, 2020
[Hemophagocytic lymphohistiocytosis]
Aude Mausoleo1, Olivier Lambotte1
1AP-HP, GHU Paris Saclay, hôpital Bicêtre, service de médecine interne-immunologie clinique, Le Kremlin-Bicêtre, France. Université Paris-Saclay, Inserm, CEA, UMR1184, Le Kremlin-Bicêtre, France.
None:
HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS. Hemophagocytic lymphohistiocytosis (HLH) is an uncontrolled, life-threatening inflammation caused by immune dysregulation and excessive activation of macrophages and T lymphocytes. HLH is characterized by persistent fever, hepatosplenomegaly, hyperferritinemia, cytopenia, coagulopathy, and hemophagocytosis. A distinction is made between the primary (genetic/familial) form (HLHp), which is caused by mutations in genes involved in the T and NK cells cytotoxicity pathway, and the secondary (acquired) form (HLHs), which usually occurs in the setting of an immunodeficiency associated with a neoplastic, infectious or autoimmune pathology. Early diagnosis and prompt treatment of the triggering factor, combined with HLH control thanks to corticosteroid therapy and sometimes immunosuppressants (etoposide, biotherapies), are the basis of management.
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