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KMID : 0616620020080040475
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Journal of Soonchunhyang Medical College
2002 Volume.8 No. 4 p.475 ~ p.479
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A Case of Letterer-Siwe Disease
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Abstract
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Letterer-Siwe disease, or acute disseminated histiocytosis X usually occurs in infants. The prognosis generally is serious, particularly if the disease is extensive.
The most common manifestations are fever, anemia, thrombocytopenia, enlargement of the liver and spleen, lymphadenopathy and pulmonary infiltrations. Osteolytic lesions are uncommon.
In about 80% of the cases, cutaneous lesions are present. They often are the first sign of the disease and are therefore of considerable diagnostic importance.
This case occured in a 14 month-old-male showing hepatosplenomegaly, dyspnea and hemorrhagic crused papular eruptions on the entire body.
The diagnosis was confirmed by clinical symptoms, laboratory data, histopathologic and. radiologic findings.
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