| Three Cases of the Letterer-Siwe Disease. |
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S H Park, Y C Song, S J Moon, K S Lee |
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Dept. of Pediatrics, School of Medicine, Han YANG University. |
| Letterer-Siwe 씨 병의 3예 보고 |
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박선희, 송영철, 문수지, 이근수 |
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한양대학교 의과대학 소아과학교실 |
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| Abstract |
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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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