Research advances in the pathogenesis, diagnosis and treatment of hemophagocytic lymphohistiocytosis in children / 中国实验血液学杂志
Journal of Experimental Hematology
; (6): 1162-1166, 2014.
Article
em Zh
| WPRIM
| ID: wpr-302327
Biblioteca responsável:
WPRO
ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH), or hemophagocytic syndrome (HPS), is characterized clinically by abrupt onset and progressive deterioration and even death. HLH is much more prevalent in children, and is potentially fatal if early diagnosis is not made and appropriate HLH-directed therapy not instituted. Increasing genetic defects and underlying diseases or causative factors have been identified to be closely implicated in the pathogenesis of HLH. In addition, great advances have been made in the past few years in terms of HLH diagnosis and clinical management. In the present review, the cause of disease, contemporary classification, epidemiology, genetic defects and molecular mechanisms, updated diagnostic criteria and novel treatment strategies for childhood HLH are summarized.
Texto completo:
1
Base de dados:
WPRIM
Assunto principal:
Patologia
/
Terapêutica
/
Diagnóstico
/
Linfo-Histiocitose Hemofagocítica
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Prognostic_studies
/
Screening_studies
Limite:
Child
/
Humans
Idioma:
Zh
Revista:
Journal of Experimental Hematology
Ano de publicação:
2014
Tipo de documento:
Article