Progress of X-linked inhibitor of apoptosis deficiency in hemophagocytic lymphohistiocytosis / 国际儿科学杂志
International Journal of Pediatrics
; (6): 145-149, 2023.
Article
en Zh
| WPRIM
| ID: wpr-989054
Biblioteca responsable:
WPRO
ABSTRACT
Hemophagocytic lymphohistiocytosis(HLH)is a systemic hyperinflammation syndrome.XIAP deficiency is an innate immunodeficiency disorder, often combined with HLH, with increased susceptibility to EB virus, mostly in childhood.XIAP deficiency leads to innate immune deficiency, increased apoptosis, and excessive activation of NLRP3 inflammasome, which together lead to impaired clearance of pathogens and excessive release of cytokines, resulting in HLH.The course of HLH in patients with XIAP deficiency is generally mild and the mortality rate is low, but it frequently relapses.In addition to HLH, XIAP deficiency can be combined with some auto-inflammatory disease.Hematopoietic stem cell transplantation is the only curable method and reduced-intensity conditioning is recommended.Some new targeted therapies, such as IFN-γ monoclonal antibodies, IL-18 and IL-6 antagonists may be effective, but the specific efficacy needs more study.
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1
Base de datos:
WPRIM
Idioma:
Zh
Revista:
International Journal of Pediatrics
Año:
2023
Tipo del documento:
Article