摘要：

组织细胞增生性病变分为组织细胞增生症X（朗格汉斯细胞组织细胞增生症）和非朗格汉斯细胞组织细胞增生症。前者好发于淋巴造血系统，常累及中枢神经系统。后者包括累及淋巴造血系统和（或）中枢神经系统的Rosai-Dorfman 病、噬血细胞性淋巴组织细胞增生症、欧迪海姆-奇斯特病、幼年性黄色肉芽肿和播散性黄色瘤、脉络丛黄色肉芽肿、脉络丛黄色瘤。本文简要介绍上述组织细胞增生性病变的临床病理学特点与鉴别要点。由于其在中枢神经系统的发病率低，未能引起神经外科和病理科医师的足够重视，易造成误诊和误治。因此，了解此类疾病的临床和病理学特征有助于提高其诊断与治疗水平。

关键词: 组织细胞增生症, 朗格汉斯细胞, 非朗格汉斯组织细胞, 病理学, 诊断, 鉴别

Abstract:

The histiocytosis is divided into Langerhans' cell histiocytosis (histiocytosis X) and non-Langerhans' cell histiocytosis (NLCH). The former mainly occurs in lymphatic hematopoietic tissue and often involves central nervous system (CNS). The latter includes Rosai-Dorfman disease (RDD), hemophagocytic lymphohistiocytosis (HLH), Erdheim-Chester disease (ECD), juvenile xanthogranuloma (JXG) and xanthoma disseminatum (XD), choroid plexus xanthogranuloma (CPXG), choroid plexus xanthoma (CPX), which may occur in lymphatic hematopoietic tissue and (or) CNS. This review has briefly introduced the clinicopathological characteristics and differential diagnosis of the above lesions. The incidence of these diseases is low in CNS, so they are often ignored, misdiagnosed and impertinently treated by neurosurgeons and pathologists. Therefore, understanding their clinical and pathological characteristics is conducive to improve the diagnosis and therapy.

Key words: Histiocytosis, Langerhans cells, Non-Langerhans histiocytes, Pathology, Diagnosis, differential