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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... |
