具有弥漫性大B细胞淋巴瘤和经典型霍奇金淋巴瘤中间特点的灰区淋巴瘤临床病理特征

Grey zone lymphoma with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma：clinicopathologic characterization of 16 cases showing different patterns

目的：探讨具有弥漫性大B细胞淋巴瘤（DLBCL）和经典型霍奇金淋巴瘤（CHL）中间特点的灰区淋巴瘤的临床和病理特征，旨在深化对该类交界性B细胞肿瘤的认识。方法回顾性分析16例典型病例的临床资料、组织学形态和免疫组织化学表型。结果16例患者男女比为1.7∶1.0，平均年龄40.2岁。8例表现为外周淋巴结病灶，5例表现为纵隔受累。根据组织学形态和免疫表型特点将该组病变分为三种模式。模式1：4例，形态学上类似于CHL，但肿瘤细胞CD20弥漫强阳性；模式2：8例，形态学上类似于DLBCL，但肿瘤细胞异质性表达CD20、PAX5，同时表达CD30和/或CD15；模式3：4例，形态特点介于CHL和DLBCL之间，肿瘤细胞不同程度地表达CD20、CD30和CD15。11例受检病例中，6例肿瘤细胞表达EB病毒潜伏膜蛋白1。临床上，绝大多数患者对联合利妥昔单抗和CHOP（ R-CHOP）方案的免疫化疗不敏感。结论通过描述三种常见的组织学模式，提出了具有DLBCL和CHL中间特点的灰区淋巴瘤的诊断标准。这类肿瘤外周型病变和纵隔病变的病理特点似有不同。目前对这组交界性肿瘤尚无有效治疗方法，患者预后较差。

Objective To profile the clinicopathologic features of a series of grey zone lymphoma （GZL） cases with hybrid features of diffuse large B-cell lymphoma （ DLBCL） and classical Hodgkin lymphoma （CHL）, with a purpose to gain an in-depth understanding of the borderline B-cell neoplasm. Methods The clinical, morphologic and immunophenotyical characteristics of 16 cases were retrospectively analyzed.Results The patients were mostly male adults , with a male to female ratio of 1.7∶1.0 and a mean age of 40.2 years.Eight patients presented with peripheral nodal lesions and five cases with mediastinal involvement .Histologically and immunophenotypically , the 16 cases were classified into three sub-categories.In 4 cases, the morphologic features resembled CHL more closely , but the neoplastic cells showed uniform and intense positive staining of CD 20 （ pattern 1 ） .Although the initial impression of the other 8 cases was that of DLBCL , the expression levels of CD 20 and PAX5 were variable , and CD30 or CD15 was positive （pattern 2）.A characteristic feature of pattern 3, observed in the remaining 4 cases, demonstrated a broad spectrum of morphology with hybrid features of both CHL and DLBCL .The neoplastic cells in pattern 3 were positive for CD20, CD30 and CD15.EBV-LMP1 was detected in 6 of the 11 tested cases.Clinically, most patients with GZL seemed insensitive to immuno-chemotherapy of the R-CHOP regimen.Conclusions The diagnostic criteria for GZL with features intermediate between DLBCL and CHL is proposed by the three histologic patterns commonly seen in these lesions .Cases presented with peripheral lesions might differ from those with mediastinal presentation pathologically .At current time , there is no＆amp;nbsp;effective treatment for these borderline B-cell lymphomas and the prognosis is poor .