边缘区淋巴瘤诊疗的现状调查分析及思考  

Analysis and reflections on the current status of diagnosis and treatment of marginal zone lymphoma

作　　者：颜文哲 向茜茜 崔国惠[3] 胡豫[3] 吕奔 张曦 彭宏凌[1] Yan Wenzhe;Xiang Xixi;Cui Guohui;Hu Yu;Lyu Ben;Zhang Xi;Peng Hongling(Department of Hematology,the Second Xiangya Hospital,Central South University,Changsha 410011,China;Medical Center of Hematology,Xinqiao Hospital of Army Medical University,Chongqing 400037,China;Institute of Hematology,Union Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan430022,China)

机构地区：[1]中南大学湘雅二医院血液内科,长沙410011 [2]陆军军医大学第二附属医院血液病医学中心,重庆400037 [3]华中科技大学同济医学院附属协和医院血液科,武汉430022

出　　处：《中华医学杂志》2024年第48期4360-4365,共6页National Medical Journal of China

摘　　要：为了了解边缘区淋巴瘤(MZL)在我国各级医院诊疗现状以及存在的问题并提出建议,中西南惰性淋巴瘤联盟在全国多家医疗机构开展多中心问卷调查,采用线上标准化问卷调查和面对面访谈相结合的方法进行调查。结果发现:(1)诊断认知上,病理医师比临床医师更重视黏膜相关淋巴组织结外MZL(MALT)与慢性炎症(86.3%与32.1%,P<0.01)以及浆细胞肿瘤(51.8%与23.1%,P<0.01)的鉴别。21%的病理医师从未行B细胞基因重排,67.6%的临床医师和69.0%的病理医师不会建议患者行脾脏穿刺来诊断脾MZL(SMZL)。(2)病理医师在脾脏穿刺标本中能确诊SMZL的平均比例仅4.1%。在治疗指征方面,40.0%的临床医师误认为Ⅲ~Ⅳ期也是治疗指征,73.0%的临床医师对治疗指征表示存在过困惑。(3)分期及疗效评估中,63.3%的医师会为患者进行PET-CT检测。(4)治疗选择上,对于具备放疗指征的患者中仅有32.2%被推荐了放疗,血液科医师选择放疗的比例明显低于肿瘤科医师:42.6%与71.7%(P<0.01);在抗幽门螺杆菌(Hp)适应证中53.1%的医师无论Hp阳性与否都会行抗Hp治疗;针对进展期MZL,临床医师一线选择免疫化疗和靶向治疗比例为62.7%与37.3%。(5)15.3%的临床医师认为现有的预后评估系统不能指导MZL的治疗方案选择。为此,作者针对MZL诊疗过程中上述现状调查结果的5个问题,从MZL的分期与疗效评估、治疗选择、预后评估等,整合多中心临床数据、开展临床研究和真实世界数据分析,以及MZL分子标记、分子亚型等,提出分析与思考。The current study aimed to understand the current status and problems of marginal zone lymphoma(MZL)in the diagnosis and treatment of hospitals at all levels in China.A multi-center questionnaire survey was conducted in a number of medical institutions across the country.A combination of online questionnaire survey and face-to-face interview was adopted.(1)Pathologists paid more attention than clinicians to distinguish MALT from chronic inflammation(86.3%vs 32.1%,P<0.01)and plasma cell tumor(51.8%vs 23.1%,P<0.01).A total of 21%pathologists never performed B-cell gene rearrangement,67.6%of clinicians and 69.0%of pathologists would not recommend splenic puncture to diagnose SMZL.(2)In terms of treatment indications,40.0%of clinicians mistakenly believed that stageⅢ-Ⅳwas also a treatment indication.Seventy percent of clinicians reported confusion about treatment indications.(3)In staging and efficacy evaluation,63.3%of physicians performed PET-CT testing for patients,mainly for resolving clinical staging(89.0%),identifying histologically transformation(79.6%),and determining the site of radiotherapy or biopsy(66.2%).(4)In terms of treatment choice,only 32.2%of patients with indications were recommended for radiotherapy,and the proportion of hematologists choosing radiotherapy was significantly lower than that of oncologists(42.6%vs 71.7%,P<0.01);In the anti-HP indications,53.1%of physicians will perform anti-HP therapy regardless of Hp positive or not;For advanced MZL,first-line immunochemotherapy was selected by 62.7%of clinicians,compared with 37.3%for targeted therapy.(5)15.3%of clinicians believed that the current prognostic evaluation system could not guide the selection of treatment options after initial treatment and recurrence.At present,there are still some cognitive deviations in the disease cognition and treatment indication of MZL among clinicians at all levels of hospitals in China.Likewise,there are still many unmet needs in MZL staging,efficacy evaluation,treatment selection and prognosis evaluati

关 键 词：边缘区淋巴瘤 现状分析 诊疗 

分 类 号：R73[医药卫生—肿瘤]