Letterer-Siwe disease: a study of thirteen cases over a 21-year period

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Abstract
In Northern Ireland, UK, with a population of 1.5 million, 13 cases of Letterer-Siwe disease were diagnosed over the past 21 yr. The average age of onset was 10.5 mo. There was a 69% mortality with an average survival time from diagnosis to death of 3.3 mo. Four children survived with no morbidity. All deaths were from pulmonary complications, but 2 cases with pulmonary infiltration responded to treatment with quadruple chemotherapy. There were no familial cases in this study.

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