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Korean J Pediatr 1994 September;73(9) :1279-1285.
Familial Hemophagocytic Lymphohistiocytosis
Dong Un Kim (Kim DU)1, Dae Kyun Koh (Koh DK)1, Yeon Dong Lee (Lee YD)1, Jae Kyun Hur (Hur JK)1, Kyoo Hong Cho (Cho KH)1, Suk Jin Kang (Kang SJ)2
1Department of Pediatrics, Catholic University Medical College, Seoul, Korea
2Department of Clinical Pathology, Catholic University Medical College, Seoul, Korea
Copyright © 1994 by The Korean Pediatric Society
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 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.
Keywords: Familial Hemophagocytic Lymphohistiocytosis
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