Familial Hemophagocytic Lymphohistiocytosis
Journal of the Korean Pediatric Society
;
: 1279-1285, 1994.
Artigo
em Coreano
| WPRIM
| ID: wpr-68629
ABSTRACT
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare disease characterized by fever, hepatosplenomegaly, cytopenia and non-malignant lymphohistiocytic infiltration with hemophagocytosis in reticulendothelial organs. We experienced three cases of FHL in identical male twins and their younger brother who presented with fever and severe hepatosplenomegaly. Cytopenia, elevated serum transaminase and low serum albumin levels, hypertriglyceridemia were common laboratory findings of them. One of them showed markedly decreased phytohemagglutinin induced lymphocyte proliferation and reversed CD4/CD8 ratio (0.52) in flowcytometric lymphocyte subset analysis. Aspirate of bone marrow revealed typical features consistent with FHL in two of them. In spite of recent therapeutic approaches, none of them survived.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Medula Óssea
/
Albumina Sérica
/
Linfócitos
/
Hipertrigliceridemia
/
Subpopulações de Linfócitos
/
Doenças Raras
/
Irmãos
/
Linfo-Histiocitose Hemofagocítica
/
Febre
Limite:
Humanos
/
Masculino
Idioma:
Coreano
Revista:
Journal of the Korean Pediatric Society
Ano de publicação:
1994
Tipo de documento:
Artigo
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