All issues > Volume 23(6); 1980

Case Report

J Korean Pediatr Soc. 1980;23(6):501-506. Published online June 15, 1980.

Three Cases of the Letterer-Siwe Disease.

S H Park, Y C Song, S J Moon, K S Lee

¹Dept. of Pediatrics, School of Medicine, Han YANG University.

Abstract

Letterer-Siwe disease is a variant of the three diseases exhibiting similar basic histology and entitled histiocysis X. Eosinophilic granuloma of bone and Hand-Schuller-Christan disease complete the triad. Letterer-Siwe disease is a clinieal-pathologic syndrome characterized by onset in infancy and by generalized hyperplasia of nonlipid-storing macrophages in liver, spleen, Lymph nodes, skin and bone marrow, which usually results in fatal outcome. During past 4 years, we experienced three cases of the disease and one of them have expired 4 monthes after the diagnosis established. But, remaining 2 cases were not fully followed-up.

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