| Three Cases of the Letterer-Siwe Disease. |
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M H Kim, E K Choi, M Y Chung, D H Keum |
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Dept, of Pediatrics, Korea General Hospital |
| Letterer-Siwe 씨 병의 3례 |
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김미향, 최은경, 정미영, 금동혁 |
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고려병원 소아과 |
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| Abstract |
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Letterer-Siwe disease was an established entity with the following fature: occurrence in infancy, hepatosplenomegaly, lymphadenopathy, bleeding diathesis, tumors of bone, generalized hyperplasia of nonlipid storing macrophages in many organs but prominently in skin, and rapidly fatal downhill course.
The diognosis was confirmed by clinical symptoms, laboratory data, histopathologic and radiologic findings.
During past 6 years, we experienced 3 cases of the disease and 2 of them expired 4 months after the diagnosis established, but the another 1 case was not fully followed-up. A brief review of literature was made.
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| Key Words:
Letterer-Siwe Disease |
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