Juvenile xanthogranuloma associated with neurofibromatosis 1: 14 patients without evidence of hematologic malignancies
Juvenile xanthogranuloma associated with neurofibromatosis 1: 14 patients without evidence of hematologic malignancies
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DOI：
10.1111/j.0736-8046.2004.21201.x
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发表时间：
2004-03-01
影响因子：
1.5
通讯作者：
Caputo, R
中科院分区：
文献类型：
--
作者：
Cambiaghi, S;Restano, L;Caputo, R
报告14例小儿神经纤维瘤病1型（NF1）的临床特点和自然病史。其中11例患者的平均随访时间为4.3年（1-10年）。在此期间，没有儿童出现恶性血液病。在13例患者中，JXG发病在生命的前2年。在这个系列中，JXG与六个或更多直径大于5毫米的咖啡斑点之间的关联是生命最初几年NF1的良好标志。总的来说，这些患者的JXG没有表现出与“经典”JXG不同的任何特征。
The clinical features and natural history of juvenile xanthogranuloma (JXG) in 14 children affected by neurofibromatosis 1 (NF1) are reported. Mean follow-up in 11 of these patients was 4.3 years (range 1-10 years). None of the children developed hematologic malignancies during this period. The onset of JXG was in the first 2 years of life in 13 of the patients. In this series, the association between JXG and six or more cafe au lait spots more than 5 mm in diameter was a good marker for NF1 in the first few years of life. Overall the JXG in these patients did not show any features distinguishable from those of "classical" JXG.