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Case Report

Familial Hemophagocytic Lymphohistiocytosis

Dong Un Kim, Dae Kyun Koh, Yeon Dong Lee, Jae Kyun Hur, Kyoo Hong Cho, Suk Jin Kang

Clin Exp Pediatr. 1994;37(9):1279-1285. Published online September 15, 1994

Familial hemophagocytic lymphohistiocytosis (FHL) is a rare disease characterized by fever, hepatosplenomegaly, cytopenia and non-malignant lymphohistiocytic infiltration with hemophagocytosis in reticuloendothelial organs. We experienced three cases of FHL in identical male twins and their younger brother who presented with fever and severe hepatosplenomegaly. Cytompenia, elevated serum transaminase and low serum albumin levels, hypertriglyceridemia were common laboratory findings of them. One of...

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