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.
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