期刊
JOURNAL OF CANCER RESEARCH AND THERAPEUTICS
卷 8, 期 2, 页码 311-313出版社
MEDKNOW PUBLICATIONS
DOI: 10.4103/0973-1482.99001
关键词
Central nervous system; juvenile xanthogranuloma; langerhans cell histiocytosis
类别
Juvenile xanthogranulomatosis (JXG) is an uncommon histiocytic disorder that is usually benign and limited to the skin. The systemic form of JXG is rare and may be associated with severe morbidity and mortality especially in central nervous system (CNS) involvement. Here, we describe a six-year-old boy with disseminated skin lesions and neurological signs and symptoms. Diagnostic work up revealed multiple brain lesions. A skin biopsy and a stereotactic brain biopsy considered suggestive of systemic JXG. Treatment with prednisolone, vinblastine and methotrexate was successful with regression of skin and CNS lesions. The patient has been in remission for almost three years.
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