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Br J Haematol ; 194(4): 745-749, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-34312844

RESUMO

BRAF inhibitors are an effective treatment for BRAFV600E -mutated, risk-organ-positive Langerhans cell histiocytosis (RO+ LCH). However, cell-free BRAFV600E DNA often persists during therapy and recurrence frequently occurs after therapy discontinuation. To identify a pathological reservoir of BRAFV600E -mutated cells, we studied peripheral blood cells obtained from six infants with RO+ multisystem (MS) LCH that received targeted therapy. After cell sorting, the BRAFV600E mutation was detected in monocytes (n = 5), B lymphocytes (n = 3), T lymphocytes (n = 2), and myeloid and plasmacytoid dendritic cells (n = 2 each). This biomarker may offer an interesting tool for monitoring the effectiveness of new therapeutic approaches for weaning children with RO+ LCH from targeted therapy.


Assuntos
Histiocitose de Células de Langerhans/tratamento farmacológico , Mutação Puntual , Inibidores de Proteínas Quinases/uso terapêutico , Proteínas Proto-Oncogênicas B-raf/antagonistas & inibidores , Proteínas Proto-Oncogênicas B-raf/genética , Criança , Pré-Escolar , Histiocitose de Células de Langerhans/sangue , Histiocitose de Células de Langerhans/genética , Humanos , Lactente , Mutação Puntual/efeitos dos fármacos , Proteínas Proto-Oncogênicas B-raf/sangue
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