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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。
Gastric Langerhans cell histiocytosis (Langerhans cell histiocytosis, LCH) is a rare disease of mononuclear macrophage system, clinical manifestations, may have multiple organ involvement, but its central nervous system complications are relatively rare. Domestic and foreign standards of diagnosis and treatment are still not uniform. This review reviewed and summarized the features of magnetic resonance imaging (MRI), neuropathological changes, clinical manifestations, risk factors and some therapeutic experiences in central LCH patients with central nervous system involvement to improve clinicians’ understanding of central nervous system complications in patients with LCH.