Progress in the Treatment of Adult Langerhans Cell Histiocytosis --Review / 中国实验血液学杂志
Journal of Experimental Hematology
;
(6): 970-974, 2022.
Artículo
en Chino
| WPRIM
| ID: wpr-939718
ABSTRACT
Langerhans cell histiocytosis (LCH) is a rare proliferative disease dominated by the proliferation of Langerhans cells, which is inflammatory myeloid neoplasms. Its clinical manifestations are variable, occurring at any age and at any site, and it is rarer in adults than in children. The gold standard for diagnosis is histopathological biopsy. Due to the rarity of adult LCH and the heterogeneity of this disease, treatment of adult LCH should be developed according to the extent of the disease and risk stratification. With the discovery of MAPK, PI3K and c-KIT signaling pathway activation, especially BRAF V600E and MAP2K1 mutations, targeted therapy has become a hot spot for therapeutic research. Meanwhile, the discovery of high expression of M2-polarized macrophages and Foxp3+ regulatory T cells (Treg) in LCH has provided an important basis for the immunotherapy. In this article, we will focus on reviewing the latest research progress in the treatment of adult LCH in recent years, and provide a reference for clinical research on the treatment of adult LCH patients.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Transducción de Señal
/
Histiocitosis de Células de Langerhans
/
Linfocitos T Reguladores
/
Proteínas Proto-Oncogénicas B-raf
/
Mutación
Límite:
Adulto
/
Niño
/
Humanos
Idioma:
Chino
Revista:
Journal of Experimental Hematology
Año:
2022
Tipo del documento:
Artículo
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