中枢神经系统白血病的发病机制及诊疗现状

Current pathogenesis,diagnosis and treatment of central nervous system leukemia

目的通过对中枢神经系统白血病(CNSL)的发病机制、诊断及治疗的新进展进行综述,提高临床医师对CNSL的认识,从而积极治疗和预防CNSL.方法使用PubMed、中国知网数据库检索系统,以"中枢神经系统白血病、发病机制、诊断、治疗、文献综述"等为中文关键词,"central nervous system leukemia,pathogenesis,diagnosis,treatment,literature review"为英文关键词进行检索.纳入标准:(1)CNSL的发病机制;(2)CNSL新的诊断方法;(3)CNSL新的治疗方案,包括分子靶向治疗和免疫治疗.排除标准:(1)数据陈旧文献;(2)内容相似或重复.最终共纳入49篇文献.结果CNSL患者的检出率随着流式细胞术免疫表型、影像学检查、脑脊液中生物标志物及组学的研究进展较前明显提高,缓解率随着新治疗方案(如分子靶向治疗和免疫治疗)的出现显著改善,然而尚未大规模开展,仍需临床试验证实.结论分子靶向治疗及免疫治疗对CNSL患者是有前景的治疗方案,但需要大量研究来验证和补充现有的临床和临床前数据,证实其安全性和有效性,改善患者预后.

Objective To review the new progress in the pathogenesis,diagnosis and treatment of central nervous system leukemia(CNSL),so as to improve clinicians'understanding of CNSL and actively treat and prevent CNSL.Methods PubMed and China knowledge Network database retrieval system were used to search with"CNSL,pathogenesis,diagnosis,treatment,literature review"as keywords.Inclusion criteria:(1)pathogenesis of CNSL;(2)new diagnostic methods of CNSL;(3)new treatment regimens for CNSL,including molecular targeted therapy and immunotherapy.Exclusion criteria:(1)obsolete data;(2)similar or repetitive contents.Finally,a total of 49articles were included.Results The detection rate of CNSL patients is significantly improved with the progress of flow cytometry immunophenotyping,imaging examination,biomarkers and histology in cerebrospinal fluid,and the remission rate is significantly improved with the emergence of new treatment regimens(such as molecular targeted therapy and immunotherapy).However,it has not been carried out on a large scale and still needs to be confirmed by clinical trials.Conclusion Molecular targeted therapy and immunotherapy are promising treatments for patients with CNSL,but a large number of studies are needed to verify and supplement the existing clinical and preclinical data,confirm their safety and effectiveness,and improve the prognosis of patients.