Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin
Adv Rheumatol
; 64: 280, 2024. tab, graf
Article
em En
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LILACS-Express
| LILACS
| ID: biblio-1556789
Biblioteca responsável:
BR1.1
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.
Texto completo:
1
Base de dados:
LILACS
Idioma:
En
Revista:
Adv Rheumatol
/
Advances in rheumatology (Online)
Assunto da revista:
Artrite
/
Reumatologia
Ano de publicação:
2024
Tipo de documento:
Article
País de afiliação:
Brasil