Histiocytic disorders are rare diseases defined by the clonal accumulation of a macrophage or dendritic cell origin. These disorders include Langerhans cell histiocytosis, Erdheim–Chester disease, juvenile xanthogranuloma, malignant histiocytoses, and Rosai–Dorfman–Destombes disease. These histiocytic disorders are a diverse group of disorders with different presentations, management, and prognosis. This review focuses on these histiocytic disorders and the role of pathological ERK signaling due to somatic mutations in the mitogen­-activated protein kinase (MAPK) pathway. Over the last decade, there has been growing awareness of the MAPK pathway being a key driver in many histiocytic disorders, which has led to successful treatment with targeted therapies, in particular, BRAF inhibitors and MEK inhibitors.

组织细胞疾病是由巨噬细胞或树突细胞起源的克隆积累定义的罕见疾病。这些疾病包括朗格汉斯细胞组织细胞增多症、埃尔德海姆-切斯特病、幼年型黄色肉芽肿、恶性组织细胞增多症和罗赛-多夫曼-德斯托姆病。这些组织细胞疾病是一组不同的疾病，具有不同的表现、治疗和预后。本综述重点关注这些组织细胞疾病以及丝裂原激活蛋白激酶 (MAPK) 通路体细胞突变导致的病理性 ERK 信号传导的作用。在过去的十年中，人们越来越认识到 MAPK 通路是许多组织细胞疾病的关键驱动因素，这导致了靶向疗法（特别是 BRAF 抑制剂和 MEK 抑制剂）的成功治疗。