摘要目的:探讨结节硬化型2级经典霍奇金淋巴瘤(cHL-NS2)的病理特征及预后。方法:回顾性收集2008年7月至2019年4月于中国医学科学院肿瘤医院治疗的cHL-NS2患者(23例)的临床病理资料，以同期55例结节硬化型1级经典霍奇金淋巴瘤(cHL-NS1)作为对照组，生存曲线采用Kaplan-Meier法绘制，预后影响因素分析采用Cox回归模型。结果:23例cHL-NS2患者的发病中位年龄为30岁，淋巴结结外受侵5例，Ann Arbor分期为Ⅰ~Ⅱ期19例。cHL-NS2肿瘤镜下病理形态表现为淋巴结结构完全破坏，淋巴结被粗大的胶原分割成结节状，胶原分割的结节内肿瘤细胞丰富，聚集成片，细胞间界限不清。cHL-NS2组患者出现肿瘤病灶坏死的比例为43.5%(10/23)，高于cHL-NS1组(18.2%，10/55)，差异有统计学意义( P＝0.040)。cHL-NS2组患者的3年无进展生存率为58.1%，低于cHL-NS1组(89.7%， P＝0.002)。全组患者中，未获得完全缓解(CR)患者3年无进展生存率为67.1%，低于获得CR患者(92.2%， P＝0.030)。多因素分析显示，cHL-NS2和一线治疗未获CR为cHL-NS患者无进展生存预后差的独立影响因素(均 P<0.05)。 结论:cHL-NS2镜下表现为肿瘤细胞形态多样，容易出现病灶坏死。在目前ABVD(多柔比星+博来霉素+长春花碱+达卡巴嗪)或BEACOPP(博来霉素+依托泊苷+多柔比星+环磷酰胺+长春新碱+丙卡巴嗪+泼尼松)一线方案治疗下，cHL-NS2为无进展生存预后差的独立影响因素。

abstractsObjective:To investigate the pathological characteristics and clinical prognosis of nodular sclerosis grade 2 of classic Hodgkin′s lymphoma (cHL-NS2) in our cancer center.Methods:A retrospective collection of 23 cases of cHL-NS2 admitted in Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College from July 2008 to April 2019 was performed. Fifty-five cases of nodular sclerosis grade 1 of classical Hodgkin′s lymphoma (cHL-NS1) during the same period were selected as control group. Survival curves were plotted using the Kaplan-Meier method, and Cox regression model was used to analyze the influencing factors for survival.Results:The median age of 23 cases of cHL-NS2 was 30 years old. Five cases had extra nodal invasion, and 19 cases were Ⅰ-Ⅱ stage based on Ann Arbor system. The pathological morphology of cHL-NS2 showed that the lymph node structure was completely destroyed and was divided into nodules by thick collagen. The tumor cells in the nodules were abundant and proliferated in sheets. The boundaries between the tumor cells were not clear. The incidence of tumor necrosis in cHL-NS2 was 43.5% (10/23), which was significantly higher than 18.2% (10/55) in cHL-NS1 ( P=0.040). The 3-year progression-free survival (PFS) rate of patients in the cHL-NS2 group was 58.1%, which was significantly lower than 89.7% in the cHL-NS1 group ( P=0.002). In all of 78 cases, the 3-year PFS rate of patients who did not obtain complete response (CR) was 67.1%, which was significantly lower than 92.2% in patients who achieved CR ( P=0.030). Multivariate Cox regression analysis demonstrated that both cHL-NS2 and failure to obtain CR by first-line treatment were independent indicators for short PFS time ( P<0.05). Conclusions:In cHL-NS2, the morphology of tumor cells are diverse, and tumor necrosis can be easily found. Under the current first-line treatments of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) or bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone (BEACOPP), cHL-NS2 is an independent indicator for worse PFS.