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A Case of Letterer-Siwe Disease.

Journal of the Korean Pediatric Society 1987;30(3):335-341.
Published online March 31, 1987.
A Case of Letterer-Siwe Disease.
Suh Hong Ha1, Jeong Sil Han1, Sung Won Kim1, Kyung Tae Kim1, Kil Hyun Kim1, Chung Hee Chi2
1Department of Pediatrics, St. Benedict Hospital, Busan, Korea
2Department of Pathology, St. Benedict Hospital, Busan, Korea
Letterer-Siwe 씨 병 1 례
하성홍1, 한정실1, 김성원1, 김경태1, 김길현1, 지정희2
1부산성분도병원 소아과
2부산성분도병원 병리과
Letterer-Siwe disease is a variant of Histiocytosis X and characterized by onset in infancy, hepatosplenomegaly, lymphadenopathy, bleeding tendency, hypochromic anemia, fever, localized bone defect and fatal outcome. The diagnosis was confirmed by clinical symptoms, laboratory datas, histopathologic and radiologic findings. We experienced a case which was treated by single prednisolone method and resulted to good response. A brief review of literature was made.
Key Words: Letterer-Siwe disease.

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