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KMID : 0367419940370091279
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Journal of Korean Pediatric Society
1994 Volume.37 No. 9 p.1279 ~ p.1285
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Familial Hemophagocytic Lymphohistiocytosis
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김동언
고대균/이연동/허재균/조규홍
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Abstract
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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.
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KEYWORD
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