J Korean Pediatr Soc.
1980 Jun;23(6):501-506.
Three Cases of the Letterer-Siwe Disease
- Affiliations
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- 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.
