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原发性皮肤大B细胞淋巴瘤:形态学、临床表现、免疫组化标志物与生存之间的关系

Primary cutaneous large B-cell lymphoma: the relation between morphology, clinical presentation, immunohistochemical markers, and survival.

作者信息

Fernández-Vázquez A, Rodríguez-Peralto J L, Martínez M A, Platón E M, Algara P, Camacho F I, López-Ríos F, Zarco C, Sánchez-Yus E, Fresno M F, Barthe L, Aliaga A, Fraga M, Forteza J, Oliva H, Piris M A

机构信息

Programa de Patología Molecular, Centro Nacional de Investigaciones Oncológicas, Madrid, Spain.

出版信息

Am J Surg Pathol. 2001 Mar;25(3):307-15. doi: 10.1097/00000478-200103000-00004.

Abstract

The histogenesis, morphology, immunophenotype, and clinical behavior of cutaneous large B-cell lymphomas (CLBCL) are largely a matter of controversy. We performed an investigation to determine whether CLBCL have features that differentiate them from other large B-cell lymphomas and whether CLBCL is itself a heterogeneous group. To this end, we reviewed the main characteristics of a series of 32 cases of LBCL found in the skin. We reviewed the clinical findings and paraffin sections of the tumors from these 32 patients. The immunohistochemical study performed included p53, MIB1, Bcl2, Bcl6, and CD10 markers. We carried out statistical analysis of these data (univariate and multivariate), seeking an association between the features of the tumors and clinical outcome, as defined by failure-free survival time. Only one patient died as a consequence of the lymphoma. Nevertheless, the accumulated probability of survival without failure at 48 months was 0.46. The number, type, and localization of the lesions were not associated with variations in either survival or failure-free survival. The expression of p53 was negative in this group of CLBCL, whereas Bcl-2 expression or localization in the lower leg did not relate to any other significant feature. Histologic examination of the cases disclosed three different groups: Grade III follicular lymphomas (FLs), monomorphous large B-cell lymphomas (LBCL type I), and LBCL with an admixed component of small B-lymphocytes (LBCL type II). Grade III FL (11 cases) tended to be found in the head and neck and showed CD10 expression in a majority of cases. A higher probability of lymph node relapses was associated with cases located in the head and neck and with CD10+ tumors. Cutaneous large B-cell lymphomas are indolent tumors, but follow an insidious course. Our data support the interpretation that CLBCL is a heterogeneous condition; comprises some LBCL derived from CD10+ germinal center cells which manifests more frequently as tumors in the head and neck region, with an increased probability of relapse in lymph nodes [1] and has some distinctive morphologic features. The existence of a component of small B-cells within the other CLBCL could lend support to the theory that some of these tumors, more than arise de novo, may have originated in preexistent small B-cell lymphomas, but no firm evidence of this is provided in this study.

摘要

皮肤大B细胞淋巴瘤(CLBCL)的组织发生、形态学、免疫表型及临床行为在很大程度上存在争议。我们进行了一项研究,以确定CLBCL是否具有使其有别于其他大B细胞淋巴瘤的特征,以及CLBCL本身是否为一个异质性群体。为此,我们回顾了在皮肤中发现的一系列32例大B细胞淋巴瘤(LBCL)的主要特征。我们回顾了这32例患者的临床发现及肿瘤的石蜡切片。所进行的免疫组化研究包括p53、MIB1、Bcl2、Bcl6和CD10标记物。我们对这些数据进行了统计分析(单变量和多变量分析),寻找肿瘤特征与临床结局之间的关联,临床结局由无瘤生存时间定义。仅1例患者死于淋巴瘤。然而,48个月时无瘤生存的累积概率为0.46。病变的数量、类型及部位与生存或无瘤生存的变化均无关联。在这组CLBCL中,p53表达为阴性,而Bcl-2在小腿的表达或定位与任何其他显著特征均无关联。对这些病例的组织学检查发现了三个不同的组:III级滤泡性淋巴瘤(FLs)、单形性大B细胞淋巴瘤(I型LBCL)和伴有小B淋巴细胞混合成分的LBCL(II型LBCL)。III级FL(11例)倾向于发生在头颈部,且大多数病例显示CD10表达。头颈部的病例及CD10+肿瘤发生淋巴结复发的可能性更高。皮肤大B细胞淋巴瘤为惰性肿瘤,但病程隐匿。我们的数据支持这样一种解释,即CLBCL是一种异质性疾病;包括一些源自CD10+生发中心细胞的LBCL,其更常表现为头颈部的肿瘤,淋巴结复发概率增加[1],且具有一些独特的形态学特征。其他CLBCL中存在小B细胞成分可能支持这样一种理论,即这些肿瘤中的一些并非新发,可能起源于先前存在的小B细胞淋巴瘤,但本研究未提供对此的确凿证据。

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