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Letterer-Siwe disease is a variant of Histiocytosis X and characterized by onset in infancy,
hepatosplenomegaly, lymphadenopathy, bleeding tendency, hypochromic anemia, fever, localized
bone defect and fatal outcome. The diagnosis was confirmed by clinical symptoms, laboratory datas,
histopathologic and radiologic findings.
We experienced a case which was treated by single prednisolone method and resulted to good
response.
A brief review of literature was made.
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