期刊
ONCOTARGETS AND THERAPY
卷 10, 期 -, 页码 521-526出版社
DOVE MEDICAL PRESS LTD
DOI: 10.2147/OTT.S121615
关键词
vemurafenib; treatment; Erdheim-Chester disease; Langerhans cell histiocytosis; pediatric histiocytic disorders
Erdheim-Chester disease (ECD) is a rare histiocytic disorder, characterized by the xanthomatous infiltration of tissues by CD68-positive and CD1a-/CD100-negative foamy histiocytes. In childhood, ECD is exceptionally rare, and only a dozen cases have been published so far. The cooccurence of Langerhans cell histiocytosis (LCH) and ECD is even rarer. Here, we report a 2-year-old boy, the youngest patient in the literature so far, who was diagnosed with concomitant BRAF mutation-positive LCH and ECD. In his case, conventional LCH treatment proved to be ineffective, but he is the youngest patient who was successfully treated with the BRAF inhibitor vemurafenib.
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