摘要目的 探讨肿瘤细胞丰富的混合细胞型经典霍奇金淋巴瘤(MCCHL-R)的临床和病理特征,并探讨预后情况.方法 回顾性分析54例MCCHL-R[肿瘤细胞数量大于10%的混合细胞型经典霍奇金淋巴瘤(MCCHL)]患者的临床病理特点,观察其组织形态学特征、免疫表型及EB病毒感染状态.同时,选取同期65例普通MCCHL进行对比分析,以观察两组患者生存率差异.结果 54例MCCHL-R占经典霍奇金淋巴瘤的比例为3.1%(54/1721).中位年龄28.5岁(9~76岁),男女比为1.6:1.0.50.0%(27/54)的患者具有B症状.98.1%(53/54)的病例发生在淋巴结,最常见的是颈部(81.5%,44/54),纵隔和脾脏受累分别为69.2%(36/54)和24.1%(13/54).结外(非淋巴器官)受累比例为41.3%(19/46).病变淋巴结结构破坏,肿瘤细胞数量>10%,形态多样(包括各种形态的H/RS(Hodgkin and Reed-Sternberg)细胞样细胞及间变性大细胞样细胞).背景混杂以小淋巴细胞为主的反应性炎性细胞.100%的病例肿瘤细胞表达CD30和弱表达PAX5,39.0%的病例EB病毒编码的小RNA(EBER)原位杂交肿瘤细胞阳性.46例(85.2%)MCCHL-R获得随访,中位随访32.5个月(5~128个月).Ⅰ~Ⅱ期和Ⅲ~Ⅳ期患者5年总生存率差异具有统计学意义(91.7%︰50.1%,P<0.05).5年总生存率低于普通MCCHL(65.5%:90.4%,P<0.05).单因素分析结果显示,年龄≥45岁、有结外累犯和就诊时疾病分期Ⅲ~Ⅳ期与MCCHL-R患者5年总生存率低有关(P<0.05).多因素Cox回归筛选结果显示,结外累犯是影响MCCHL-R患者的独立预后因素(RR:4.352,95%CI:1.122~16.879,P<0.05).结论 MCCHL-R多见于年轻人,病变具有霍奇金淋巴瘤的特征,但肿瘤细胞丰富(>10%),免疫表型与经典霍奇金淋巴瘤相同,较普通MCCHL预后差,结外累犯是MCCHL-R的独立预后因子.

abstractsObjective To investigate the clinicopathologic characteristics of neoplastic cell-rich mixed cellularity classical Hodgkin lymphoma( MCCHL-R) and to compare the prognosis with typical mixed cellularity classic Hodgkin lymphoma(MCCHL). Methods Fifty-four patients with MCCHL-R(the tumor cells >10%) and 65 patients with typical MCCHL identified from 1721 Hodgkin lymphomas were reviewed to compare the clinicopathological characteristics including morphologic and immunophenotypic features, EBV infection status, clinical therapy and overall survival. Results The median age of the patients of MCCHL-R was 28. 5 years(range:9-76 years, male:female=1. 6:1. 0). Twenty-seven patients(50. 0%) had B symptoms. Most patients had cervical lymph node involvement ( 81. 5%, 44/54 ) . Mediastinum and spleen involvement were seen in 69. 2%( 36/54 ) and 24. 1%( 13/54 ) , respectively. Extranodal non-lymphoid organ involvement was seen in 41. 3%( 19/46) cases. Morphologically, lymph node architectureswere effaced at various degree with large neoplastic cells of variable morphology, including Hodgkin/Reed-Sternberg ( H/RS ) cells and anaplastic large cells. There were abundant background heterogeneous admixtures of non-neoplastic inflammatory and accessory cells that were predominant mature small lymphocytes. All tumors were positive for CD30 and weakly positive for PAX5. Epstein-Barr encoded RNA ( EBER) detectable by in situ hybridization was seen in 39. 0% cases. Forty-six patients had a median follow-up time of 32. 5 months( range:5-128 months) and the 5-year survival rate for stageⅠ-Ⅱand stageⅢ-Ⅳ patients were 91. 7% and 50. 1%, respectively(P<0. 05). The 5-year survival rate for MCCHL-R was lower than typical MCCHL patients. Single factor analysis showed that age of >45 years, extranodal involvement and stage Ⅲ-Ⅳ were correlated with poorer 5-year survival rate ( P<0. 05 ) . Multiple factors Cox proportional hazards regression showed that extranodal involvement was the independent prognostic factor ( RR:4. 352, 95%CI:1. 122-16. 879, P<0. 05 ) . Conclusions MCCHL-R is more common in young people. The tumor has pathological features of classic Hodgkin lymphoma enriched with the tumor cells (>10%) and similar immunophenotype to classical Hodgkin lymphoma. Compared with typical MCCHL, extranodal disease is an independent prognostic factor of MCCHL-R.