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Familial Erythrophagocytic Lymphohistiocytosis in Siblings

Journal of the Korean Pediatric Society 1995;38(3):428-434.
Published online March 15, 1995.
Familial Erythrophagocytic Lymphohistiocytosis in Siblings
Eun Sook Lee, Ji Eun Choi, Dug Ha Kim, Hae Ran Lee, Chong Young Park
Department of Pediatrics, College of Medicine, Hallyn University, Seoul, Korea
자매에서 발병된 Familial Erythrophagocytic Lymphohistiocytosis 부검례
이은숙, 최지은, 김덕하, 이혜란, 박종영
한림대학교 의과대학 소아과학교실
Abstract
Familial erythrophagocytic lymphohistiocytosis(FEL) is an uncommon disorder characterized by multi-organ infiltration with phagocytic histiocytes and macrophages. It is a familial discorder presenting during infancy or young childhood with fever, hepartosplenomegaly, pancytopenia, bleeding diathesis, hypertriglyceridemia and neurologic manifestations. The course of the disease is extremely lethal and diagnosis of the disease during lifetime is very difficult. Exact diagnosis can only be made by autopsy. We experienced FEL cases in siblings, who had fever, hepatosplenomegly, pancytopenia, and hypertriglyceridemia. Bone marrow study showed increased histiocytes with active hemophagocytosis. They were died due to multiple organ failure, and the diagnosis was confirmed by autopsy, so we report it and review the related literatures.
Key Words: Familial Erythrophagocytic Lymphohistiocytosis, Autopsy


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