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
Department of Hematology and Oncology, Mie University Graduate School of Medicine, 2-174 Edobashi, Tsu, Mie 514-8507, Japan.
Haematologica. 2008 Aug;93(8):1195-202. doi: 10.3324/haematol.12810. Epub 2008 Jun 12.
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.
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.
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.
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.
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。
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。
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。
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。