科研之友
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摘要
objectives: to describe the clinical course of langerhans cell histiocytosis and to compare its outcome according to age, staging of the disease and treatment response. methods: retrospective analysis of data on 33 children with langerhans cell histiocytosis followed at hospital das cl赤nicas, universidade federal de minas gerais, brazil, between 1988 and 2004. results: age at diagnosis ranged from 2 months to 16 years (median: 2.5 years). seventeen children were male. the follow-up period varied from 21 days to 16.2 years (median: 3.4 years). the most common clinical manifestations at diagnosis were osteolytic lesions, enlarged lymph nodes and skin lesions. the overall survival rate for the whole group was 86.1% at 16 years (95%ci 66.6-94.6%). deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. those patients who had a %26quot;better%26quot; response to treatment in the sixth week were likely to have a significantly higher overall survival rate than those who showed disease progression. overall survival rate was significantly higher for patients with single-system disease. the disease-free survival rate for the whole group was 30.9% at 16 years (95%ci 15.6-47.5%), and was significantly higher for those with single-system disease. age groups were not associated with different disease-free survival rates. diabetes insipidus was the most common sequela. no cases of secondary neoplasms were observed. conclusion: the clinical manifestations of langerhans cell histiocytosis vary widely, with a high relapse rate and low mortality rate.
