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弥散大B细胞淋巴瘤(DLBCL)占儿童非霍奇金淋巴瘤的10%,是一组有多种不同临床和生物学特性亚型的非均一性疾病。根据WHO分类,DLBCL在形态学上可分为中心母细胞型、免疫母细胞型、富T细胞/组织细胞型、间变细胞型及非特指型等类型。与成人DLBCL相比,对CD10、BCL6和MUM-1的免疫组织化学研究显示,儿童DLBCL以生发中心型为主。鉴别诊断包括伯基特淋巴瘤、原发纵隔大B细胞淋巴瘤、前体B细胞淋巴母细胞淋巴瘤、滤泡型淋巴瘤和结性淋巴细胞优势型霍奇金淋巴瘤。现有研究表明成人和儿童DLBCL在细胞来源、遗传学异常和对治疗反应方面有所不同。新近的多中心研究显示儿童DLBCL的无事件生存率可达90%。抗CD20的单克隆抗体美罗华在儿童DLBCL治疗中的作用还有待进一步研究确定。
Diffuse large B-cell lymphoma (DLBCL), which accounts for 10% of children with non-Hodgkin’s lymphoma, is a heterogeneous group of diseases with many different clinical and biological subtypes. According to WHO classification, DLBCL morphologically can be divided into centroblastoma type, immunoblastic type, T-rich cell / tissue type, metaplastic cell type and non-specific type. Immunohistochemical studies of CD10, BCL6 and MUM-1 compared with adult DLBCL showed that children’s DLBCL predominates in germinal centers. Differential diagnoses include Burkitt’s lymphoma, primary mediastinal large B cell lymphoma, precursor B cell lymphoblastic lymphoma, follicular lymphoma, and lymph node-predominant Hodgkin lymphoma. Existing research shows that adult and pediatric DLBCL differ in terms of cellular origin, genetic abnormalities, and response to treatment. Recent multicenter studies have shown that children with DLBCL have an event-free survival rate of up to 90%. The role of anti-CD20 monoclonal antibody rituximab in the treatment of pediatric DLBCL remains to be determined further study.