Familial Hemophagocytic Lymphohistiocytosis
Journal of the Korean Pediatric Society
;
: 1279-1285, 1994.
Article
in Korean
| 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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Bone Marrow
/
Serum Albumin
/
Lymphocytes
/
Hypertriglyceridemia
/
Lymphocyte Subsets
/
Rare Diseases
/
Siblings
/
Lymphohistiocytosis, Hemophagocytic
/
Fever
Limits:
Humans
/
Male
Language:
Korean
Journal:
Journal of the Korean Pediatric Society
Year:
1994
Type:
Article
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