All issues > Volume 30(3); 1987

Case Report

J Korean Pediatr Soc. 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

Abstract

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.

Keywords :Letterer-Siwe disease.