Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin
Adv Rheumatol
;
64: 280, 2024. tab, graf
Article
Dans Anglais
|
LILACS-Express
| LILACS
| ID: biblio-1556789
ABSTRACT
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
langue:
Anglais
Texte intégral:
Adv Rheumatol
Thème du journal:
Artrite
/
Reumatologia
Année:
2024
Type:
Article
Pays d'affiliation:
Brésil
Institution/Pays d'affiliation:
Brazilian Society of Rheumatology/BR
/
Universidade Federal Fluminense/BR
/
Universidade Federal do Rio de Janeir/BR
/
Universidade Federal do Rio de Janeiro/BR
/
Universidade do Estado do Rio de Janeir/BR
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