摘要目的 探讨儿童淋巴结边缘区淋巴瘤(NMZL)的临床病理学特征.方法 观察北京友谊医院2008年1月至2018年10月收集的7例儿童NMZL的组织学形态和免疫表型,检测细胞克隆性,分析临床信息并进行随访.结果(1)7例患者均为男性,10～26岁,中位年龄15岁.7例均为局部淋巴结肿大,其中5例为颈部淋巴结,1例为耳前淋巴结,1例为耳后淋巴结,患者均无B症状.(2)形态学上,7例均表现为淋巴结内滤泡增多,大小不等,皮髓质均有分布,淋巴结的固有结构不清楚.每例中均具有突出的大滤泡,其边缘不规则,生发中心可见中心细胞和中心母细胞;滤泡边缘区及滤泡间区明显增宽,其中的细胞趋于单一化,由单核样B细胞、中心细胞样细胞和浆细胞样细胞组成;散在进行性转化的生发中心,淋巴结周边残留少量固有淋巴组织.(3)免疫表型:7例均呈CD20弥漫阳性(滤泡和滤泡间区均阳性),滤泡间区(病变区)Ki?67阳性细胞指数约30%～40%;不表达其他B细胞淋巴瘤的特定标志物.(4)7例免疫球蛋白(Ig)基因重排检测均阳性.(5)7例随访平均13个月,均存活,未见复发.结论 NMZL是一种罕见的淋巴瘤,几乎仅发生在男性儿童和青年,好发于头颈部淋巴结,具有独特的形态学及"良性"的临床过程,预后很好.

abstractsObjective To investigate the pathological features and clinical manifestation of pediatric nodal marginal zone lymphoma(NMZL).Methods Histological morphology and immunophenotype of 7 cases of pediatric NMZL were retrospectively reviewed at Beijing Friendship Hospital Affiliated to Medical University between January 2008 to October 2018. Clonal rearrangement analysis was performed. Clinical information including patient follow?up data were analyzed. Results All 7 patients were male with a median age of 15 years aged from 10 to 26 years. All patients presented with only lymph node enlargement without B symptoms, including cervical lymph node (5 cases), preauricular lymph node (1 case) and retroauricular lymph node (1 case). Histologically, all cases showed irregular large follicles on the edges with widened marginal areas and intervesicular areas, and lesional cells were uniform with progressive transformation of germinal center centers along with a small amount of intrinsic lymphoid tissue. All 7 cases showed diffuse CD20 positivity both follicle and interfollicular region along with 30%-40% positivity in the interfollicular region (pathological region). Markers of other B?cell lymphomas werenot expressed. All 7 cases were positive for immunoglobulin(Ig) gene rearrangement. None of the patients showed no recurrence up on after follow?up for an average of 13 months. Conclusions Pediatric NMZL is a rare type of lymphoma that has a unique morphology and occurs almost exclusively in male children and young adults and often in head and neck lymph nodes. It has an excellent prognosis. Therefore, awareness of the disease with accurate diagnosis is important.