中枢神经系统受累的郎格罕斯细胞组织细胞增生症诊治进展

Progress in the diagnosis and treatment of Langerhans cell histiocytosis with central nervous system involvement

胃郎格罕斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)是一种少见的单核巨噬系统疾病,临床表现多样,可有多脏器受累,但其中枢神经系统合并症相对少见。国内外对其诊断及治疗标准仍不统一。该文回顾及总结中枢神经系统受累LCH患者的磁共振成像(MRI)特点、神经病理学变化、临床表现、危险因素以及一些治疗经验,以提高临床医师对LCH患者中枢神经系统合并症的认识。

Langerbans cell histiocytosis （LCH） is a rare disease of the monocyte-macrophage system. The clinical presentation of LCH varies. It commonly has multi-organ involvement, but rarely has the involvement of central nervous system （CNS）. The standards for the diagnosis and treatment of LCH are not consistent in different countries. In this review, the features of magnetic resonanee imaging （MRI）, the neuropathologic changes, the clinical manifestation, the risk factors, and the available therapeutic experiences of LCH patients with CNS involvement were summarized, so as to improve the clinical recognition of the disease for the clinicians.