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Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin
Sztajnbok, Flavio; Fonseca, Adriana Rodrigues; Campos, Leonardo Rodrigues; Lino, Kátia; Félix Rodrigues, Marta Cristine; Silva, Rodrigo Moulin; Gasparello de Almeida, Rozana; Perazzio, Sandro Félix; Fernandes Carvalho, Margarida de Fátima.
Afiliación
  • Sztajnbok, Flavio; Universidade Federal do Rio de Janeir. Department of Pediatrics. Rio de Janeiro. BR
  • Fonseca, Adriana Rodrigues; Universidade Federal do Rio de Janeir. Department of Pediatrics. Rio de Janeiro. BR
  • Campos, Leonardo Rodrigues; Universidade Federal Fluminense. Hospital Universitário Antônio Pedro. Niterói. BR
  • Lino, Kátia; Universidade Federal Fluminense. Hospital Universitário Antônio Pedro. Niterói. BR
  • Félix Rodrigues, Marta Cristine; Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteir. Pediatric Rheumatology Division. Rio de Janeiro. BR
  • Silva, Rodrigo Moulin; Universidade do Estado do Rio de Janeir. Hospital Universitário Pedro Ernesto. Rio de Janeiro. BR
  • Gasparello de Almeida, Rozana; Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteira. Rio de Janeiro. BR
  • Perazzio, Sandro Félix; Brazilian Society of Rheumatology. Rare Diseases Committee. Rio de Janeiro. BR
  • Fernandes Carvalho, Margarida de Fátima; Brazilian Society of Rheumatology. Rare Diseases Committee. Rio de Janeiro. BR
Adv Rheumatol ; 64: 280, 2024. tab, graf
Article en En | LILACS-Express | LILACS | ID: biblio-1556789
Biblioteca responsable: 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.
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Texto completo: 1 Colección: 01-internacional Base de datos: LILACS Idioma: En Revista: Adv Rheumatol Asunto de la revista: Artrite / Reumatologia Año: 2024 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Brasil
Texto completo
Añadir a Mi BVS
Imprimir
XML
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: LILACS Idioma: En Revista: Adv Rheumatol Asunto de la revista: Artrite / Reumatologia Año: 2024 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Brasil