BRAF V600E-Positive Congenital Multisite Langerhans Cell Histiocytosis.
Autor: | Prada Avella MC; Pediatric Oncology, Instituto Nacional de Cancerología, Bogota, COL., Suárez A Sr; Pediatric Oncology, Instituto Nacional de Cancerología, Bogotá, COL., Contreras S; Pediatrics, Instituto Nacional de Cancerología, Bogotá, COL., Calderon A; Pediatric Oncology, Instituto Nacional de Cancerología, Bogotá, COL. |
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Jazyk: | angličtina |
Zdroj: | Cureus [Cureus] 2020 Sep 02; Vol. 12 (9), pp. e10200. Date of Electronic Publication: 2020 Sep 02. |
DOI: | 10.7759/cureus.10200 |
Abstrakt: | Congenital Langerhans cell histiocytosis (LCH) usually manifests as a disease limited to the skin, with self-healing characteristics; however, in some cases, it may be a more severe entity, with multisystemic expression and poor prognosis. We present the case of a patient diagnosed with multisystemic congenital LCH, with the presence of the BRAF V600E mutation, with a severe form of the disease, with risk organ compromise, and manifestations of resistance to chemotherapy. This case is a challenge due to the disease's biologically aggressive behavior in this patient. It presents unique treatment difficulties as a result of inherent resistance to conventional therapy and uncertain response to BRAF inhibitors. Competing Interests: The authors have declared that no competing interests exist. (Copyright © 2020, Prada Avella et al.) |
Databáze: | MEDLINE |
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