儿童非霍奇金淋巴瘤的临床及病理特点

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目的:对73例儿童非霍奇金淋巴瘤临床资料进行回顾性分析,明确其临床特点、病理类型及与疗效的关系。方法:收集2014年10月至2018年10月上海市儿童医院73例初诊非霍奇金淋巴瘤患儿资料,其中男52例,女21例;年龄2~13岁。对所有患儿发病的起始或累积器官、病理诊断、疾病分期、手术及疗效等进行综合评估。结果:73例儿童非霍奇金淋巴瘤中66例获得明确的病理诊断,其中38例由手术确诊,20例由活检确诊,且均通过免疫组织化学明确,8例通过流式细胞术等确诊;另7例中的6例获大致病理分类,仅1例分类不明。66例明确病例中以伯基特淋巴瘤最多为31例,其余依次为T-淋巴母细胞型14例、间变大淋巴瘤7例、B-淋巴母细胞型6例、弥漫大B淋巴瘤5例、结外鼻型自然杀伤/T细胞淋巴瘤1例、母细胞性浆细胞样树突细胞瘤1例和脂膜炎样T淋巴瘤1例;起始部位或累积器官涉及多个器官,以颈部(20例)、腹腔(15例)、纵隔(6例)等最为多见;临床转归与疗效中Ⅱ期、Ⅲ期和Ⅳ期的完全缓解率分别为100.0%、85.1%和72.2%;Ⅲ期和Ⅳ期患儿无事件生存率低于Ⅱ期组患儿(80.9%比66.7%比100.0%),病理类型分组结果显示淋巴母细胞淋巴瘤较伯基特淋巴瘤无事件生存率更低(73.7%比90.3%),但差异均无统计学意义(均n P>0.05)。n 结论:儿童非霍奇金淋巴瘤男性发病多,婴幼儿少见,病理类型较成人单纯,以伯基特淋巴瘤最多,临床分期和病理类型与预后可能相关。“,”Objective:To retrospectively analyze the clinical data of 73 children with non-Hodgkin′s lymphoma to determine its clinical features, pathological types and relationship with efficacy.Methods:In Shanghai Children′s Hospital from October 2014 to October 2018, 73 children with newly diagnosed non-Hodgkin′s lymphoma were enrolled for a retrospective study, with 52 males and 21 females, aged between 2 and 13 years old, so as to comprehensively assess cumulative organs, pathological diagnosis, stage of disease, surgery and efficacy.Results:Among the 73 children with non-Hodgkin′s lymphoma, 66 cases had a clear pathological diagnosis, including 38 cases of surgical pathology and 20 cases of biopsy pathology.All of them were confirmed by immunohistochemisty, and 8 cases were determined by flow cytometry.Meanwhile, 6 cases of the rest 7 cases obtained approximate pathological classification.Only 1 case was unknown.Among the 66 cases, Burkitt lymphoma was the most common in 31 cases, followed by 14 cases of T-lymphocyte type, 7 cases of enlarged lymphoma, 6 cases of B-lymphocyte type, 5 cases of diffuse large B lymphoma, 1 case of extranodal natural killer/T cell lymphoma-nasal type, 1 case of blastic plasmacytoid dendritic cell neoplasm, and 1 case of panniculitis-like T lymphoma.The initial site or cumulative organ involved multiple organs, with most common in neck(20 cases), abdominal cavity(15 cases), and mediastinum(6 cases). In view of outcomes and efficacy, the complete remission rates of stage Ⅱ, Ⅲ, and Ⅳ in clinical were 100.0%, 85.1%, and 72.2%, respectively.The event-free survival rates in stage Ⅲ and Ⅳ were lower than those in stage Ⅱ(80.9% n vs. 66.7% n vs. 100.0%). From the perspective of the main pathological types grouping, the results revealed that lymphoblastic lymphoma had a lower event-free survival rate than Burkitt′s lymphoma (73.7% n vs.90.3%), while there was no statistical significance(all n P>0.05).n Conclusions:Non-Hodgkin′s lymphoma in children is more common in men, and less common in infants and young children.Pathological type is simpler than adults, Burkitt lymphoma is the most common type, and clinical stage and pathological type may be related to prognosis.
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