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Familial Hemophagocytic Lymphohistiocytosis

Journal of the Korean Pediatric Society 1994;37(9):1279-1285.
Published online September 15, 1994.
Familial Hemophagocytic Lymphohistiocytosis
Dong Un Kim1, Dae Kyun Koh1, Yeon Dong Lee1, Jae Kyun Hur1, Kyoo Hong Cho1, Suk Jin Kang2
1Department of Pediatrics, Catholic University Medical College, Seoul, Korea
2Department of Clinical Pathology, Catholic University Medical College, Seoul, Korea
3형제에서 발생한 가족성 혈액탐식성 림프조직구 증식증
김동언1, 고대균1, 이연동1, 허재균1, 조규홍1, 강석진2
1가톨릭대학교 의과대학 소아과학교실
2가톨릭대학교 의과대학 임상병리학교실
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.
Key Words: Familial Hemophagocytic Lymphohistiocytosis

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