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原发性CD5+弥漫性大B细胞淋巴瘤:120例患者详细临床病理分析结果

De novo CD5+ diffuse large B-cell lymphoma: results of a detailed clinicopathological review in 120 patients.

作者信息

Yamaguchi Motoko, Nakamura Naoya, Suzuki Ritsuro, Kagami Yoshitoyo, Okamoto Masataka, Ichinohasama Ryo, Yoshino Tadashi, Suzumiya Junji, Murase Takuhei, Miura Ikuo, Ohshima Koichi, Nishikori Momoko, Tamaru Jun-ichi, Taniwaki Masafumi, Hirano Masami, Morishima Yasuo, Ueda Ryuzo, Shiku Hiroshi, Nakamura Shigeo

机构信息

Department of Hematology and Oncology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan.

出版信息

Haematologica. 2008 Aug;93(8):1195-202. doi: 10.3324/haematol.12810. Epub 2008 Jun 12.

Abstract

BACKGROUND

De novo CD5-positive diffuse large B-cell lymphoma (CD5(+) DLBCL) is clinicopathologically and genetically distinct from CD5-negative (CD5(-)) DLBCL and mantle cell lymphoma. The aim of this retrospective study was to clarify the histopathological spectrum and obtain new information on the therapeutic implications of CD5(+) DLBCL.

DESIGN AND METHOD

From 1984 to 2002, 120 patients with CD5(+) DLBCL were selected from 13 collaborating institutes. We analyzed the relationship between their morphological features and long-term survival. The current series includes 101 patients described in our previous study.

RESULTS

Four morphological variants were identified: common (monomorphic) (n=91), giant cell-rich (n=13), polymorphic (n=14), and immunoblastic (n=2). Intravascular or sinusoidal infiltration was seen in 38% of the cases. BCL2 protein expression in CD5(+) DLBCL was more frequent than in CD5(-) DLBCL (p=0.0003). Immunohistochemical analysis in 44 consecutive cases of CD5(+) DLBCL revealed that 82% of these cases (36/44) were non-germinal center B-cell type DLBCL. The 5-year overall survival rate of the patients with CD5(+) DLBCL was 38% after a median observation time of 81 months. Patients with the common variant showed a better prognosis than those with the other three variants (p=0.011), and this was confirmed on multivariate analysis. Overall, 16 patients (13%) developed central nervous system recurrence.

CONCLUSIONS

Our study revealed the morphological spectrum of CD5(+) DLBCL, found that the incidence of central nervous system recurrence in this form of lymphoma in high, confirmed that CD5(+) DLBCL frequently expresses BCL2 protein and showed that it is mainly included in the non-germinal center B-cell type of DLBCL.

摘要

背景

原发性CD5阳性弥漫性大B细胞淋巴瘤(CD5(+) DLBCL)在临床病理和基因方面与CD5阴性(CD5(-))DLBCL及套细胞淋巴瘤不同。这项回顾性研究的目的是明确CD5(+) DLBCL的组织病理学谱,并获取有关其治疗意义的新信息。

设计与方法

1984年至2002年期间,从13个合作机构中选取了l20例CD5(+) DLBCL患者。我们分析了其形态学特征与长期生存之间的关系。本系列研究包括我们之前研究中描述的101例患者。

结果

识别出四种形态学变异型:普通(单形性)(n = 91)、富含巨细胞型(n = 13)、多形性(n = 14)和免疫母细胞型(n = 2)。38%的病例可见血管内或窦状浸润。CD5(+) DLBCL中BCL2蛋白表达比CD5(-) DLBCL更常见(p = 0.0003)。对44例连续的CD5(+) DLBCL病例进行免疫组织化学分析显示,这些病例中有82%(36/44)为非生发中心B细胞型DLBCL。CD5(+) DLBCL患者在中位观察时间81个月后的5年总生存率为38%。普通变异型患者比其他三种变异型患者预后更好(p = 0.011),多因素分析证实了这一点。总体而言,16例患者(13%)发生中枢神经系统复发。

结论

我们的研究揭示了CD5(+) DLBCL的形态学谱,发现这种淋巴瘤形式中枢神经系统复发的发生率较高,证实CD5(+) DLBCL经常表达BCL2蛋白,并表明其主要属于非生发中心B细胞型DLBCL。

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