All issues > Volume 13(12); 1970

Case Report

J Korean Pediatr Soc. 1970;13(12):701-708. Published online December 31, 1970.

A Case Report of the Letterer-Siwe Disease

Y.J. Choi¹, S.W. Ahn¹, E.S. Chang¹, K.P. Choi², Y.K. Chun², M.H. Huh³

¹Dept. of Pediatrics Wallace Memorial Baptist Hospital, Pusan, Korea
²Dept. of Surgery Wallace Memorial Baptist Hospital, Pusan, Korea
³Dept.of Pathology Wallace Memorial Baptist Hospital, Pusan, Korea

Abstract

A case of Letterer-Siwe disease in 1 4/12 year old boy manifesting all of the classic signs described by Siwe-hepatosplenomegaly, swelling of the lymph nodes, bleeding tendency, hypochromic anemia, fever and osseous lesions is presented. The diagnosis was confirmed by skin and lymph node biopsies and radiological evidence of bony defects in the skull and vertebrae. Brief review and discussion were made mainly on etiological factors, secondary infections, skin rashes and other clinical signs, and histological findings. This is the second reported case in Korean literature and the first one with histological confirmation on lymph node biopsy with specific bone lesions.

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