Effective BRAF inhibitor vemurafenib therapy in a 2-year-old patient with sequentially diagnosed Langerhans cell histiocytosis and Erdheim–Chester disease
Z Váradi, R Bánusz, J Csomor, K Kállay… - OncoTargets and …, 2017 - Taylor & Francis
OncoTargets and therapy, 2017•Taylor & Francis
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 …
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 …
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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