原发性中枢神经系统淋巴瘤诊断及治疗专家共识(2024年版)

Chinese expert consensus on the diagnosis and treatment of primary central nervous system lymphoma (2024 version)

原发性中枢神经系统淋巴瘤(PCNSL)是罕见类型的结外非霍奇金淋巴瘤,病灶范围局限于脑实质、脊髓、软脑膜和眼。年发病率为(0.4~0.5)/10万。95%以上患者的病理类型为弥漫大B细胞淋巴瘤,表现为典型血管中心性生长模式。世界卫生组织(WHO)将PCNSL归类为弥漫大B细胞淋巴瘤的一个独特亚型,属于免疫豁免部位的大B细胞淋巴瘤。PCNSL是所有非霍奇金淋巴瘤中预后最差的类型,5年预估总生存率仅为30.5%。由于PCNSL临床罕见,对该疾病的认识尚未统一,诊治有待进一步规范。为加强我国临床医师对PCNSL的认知,提高诊断和治疗水平,中华医学会血液学分会淋巴细胞疾病学组、中国临床肿瘤学会(CSCO)淋巴瘤专家委员会相关专家参考国内外指南、共识并结合最新的研究进展,讨论并制定本共识。

Primary central nervous system lymphoma(PCNSL)is a rare type of extranodal non-Hodgkin lymphoma with lesions limited to the brain parenchyma,spinal cord,soft meninges and eyes.The annual incidence is(0.4-0.5)/100,000,and more than 95%of patients have diffuse large B-cell lymphoma with a typical angiocentric growth pattern.The World Health Organization(WHO)defines PCNSL as a distinct subtype of diffuse large B-cell lymphoma:large B-cell lymphoma of immune-privileged sites.PCNSL has the worst prognoses of all non-Hodgkin lymphomas,with a 5-year overall survival rate of 30.5%.Due to the clinical rarity of PCNSL,the understanding of this disease has not been unified,and the diagnosis and treatment need to be further standardized.In order to strengthen clinicians'knowledge of PCNSL and improve diagnosis and treatment,the experts from the Lymphoid Disease Group,Chinese Society of Hematology,Chinese Medical Association and Lymphoma Expert Committee of Chinese Society of Clinical Oncology(CSCO)have discussed and formulated the present consensus by referring to domestic and international guidelines or consensus and combining with the latest research progress.