间变性淋巴瘤激酶阳性组织细胞增生症的诊疗新进展  

Recent Progress in Diagnosis and Treatment of Anaplastic Lymphoma Kinase-Positive Histiocytosis

作　　者：王思佳 虞燕萍 吴怡玲 张志群 WANG Sijia;YU Yanping;WU Yiling;ZHANG Zhiqun(The Fourth School of Clinical Medicine,Zhejiang Chinese Medical University,Hangzhou 310000,China;Department of Pediatrics,the Affiliated Hangzhou First People′s Hospital,School of Medicine of Westlake Universty,Hangzhou 310000,China)

机构地区：[1]浙江中医药大学第四临床医学院,杭州310000 [2]西湖大学医学院附属杭州市第一人民医院儿科,杭州310000

出　　处：《医学综述》2025年第8期938-943,共6页Medical Recapitulate

基　　金：浙江省医药卫生科技计划项目(2023KY917)。

摘　　要：间变性淋巴瘤激酶(ALK)阳性组织细胞增生症是一种罕见的、神经系统频繁受累的组织细胞增殖性疾病,其临床表现多样,可分为单发性、多发性及系统性(多部位、器官受累)。由于报道的病例数有限,其完整的临床病理学特征和分子谱系尚未完全阐明,诊断上需结合临床影像学、病理学、免疫组织化学及必要的基因检测。国际上尚未确立其治疗的统一指导方案,目前常用的治疗方法包括支持性治疗观察、手术切除、放疗、常规全身治疗、ALK靶向治疗等。因此,探索ALK阳性组织细胞增生症的发病机制,以及开发新的治疗方法是未来的研究重点。Anaplastic lymphoma kinase(ALK)-positive histiocytosis is a rare histiocytosis with frequent nervous system involvement.Its clinical manifestations are varied,and can be divided into single,multiple and systemic(multi-site,organ involvement).Due to the limited number of reported cases,the complete clinicopathological features and molecular lineage have not been fully elucidated,and clinical imaging,pathology,immunohistochemistry and necessary genetic testing are required for the diagnosis.The international unified guidance program for its treatment has not been established.Currently,the commonly used treatment methods include supportive treatment observation,surgical resection,radiotherapy,conventional systemic therapy,ALK targeted therapy,etc.Therefore,exploring the pathogenesis of ALK-positive histiocytosis and developing new therapeutic methods are the focus of the future research.

关 键 词：间变性淋巴瘤激酶 阳性组织细胞增生症 免疫组织化学染色 放化疗 间变性淋巴瘤激酶抑制剂 

分 类 号：R725.5[医药卫生—儿科]