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.
Artículo
en Chino
| WPRIM
| ID: wpr-302327
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:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Patología
/
Terapéutica
/
Diagnóstico
/
Linfohistiocitosis Hemofagocítica
Tipo de estudio:
Estudio diagnóstico
/
Estudio de etiología
/
Estudio pronóstico
/
Estudio de tamizaje
Límite:
Niño
/
Humanos
Idioma:
Chino
Revista:
Journal of Experimental Hematology
Año:
2014
Tipo del documento:
Artículo
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