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KMID : 0378019820250020045
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New Medical Journal
1982 Volume.25 No. 2 p.45 ~ p.50
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A case of Letterer-Siwe disease
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Abstract
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Letterer-Siwe´s disease is an acute disseminated reticuloendotheliosis outlined by Siwe in 1933 and its classic diagnostic features are consisted of 1) nonfamilial disease of unknown etiology, usually occurring in infancy or early childhood and ending fatally after an illness of a few weeks´ to several months´ duration; 2) moderate to marked enlargement of liver and spleen; 3) moderately enlarged, discrete, nontender lymph nodes; 4) a hemorrhagic diathesis consisting chiefly of petechial skin lesions; 5) a progressive hypochromic anemia; 6) a low-grade temper￢ature elevation; 7) production of localized bone defects in some cases; 8) A generalized hyper￢plasia of the reticuloendothelial system involving the spleen, liver, lymph nodes, skin, bone marrow, and thymus gland.
We have experienced a case of Letterer-Siwe´s disease in 4 month old girl who had the ty￢pical features with the exception of the localized bone defects and also made brief review of the related literatures.
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