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携带细胞周期蛋白D3表达的骨髓大B细胞淋巴瘤:7例患者的临床、形态学、免疫表型和基因分析

Bone marrow large B cell lymphoma bearing cyclin D3 expression: clinical, morphologic, immunophenotypic, and genotypic analyses of seven patients.

作者信息

Watanuki Jyuri, Hatakeyama Kinta, Sonoki Takashi, Tatetsu Hiro, Yoshida Katsuhiko, Fujii Soichiro, Mizutani Minoru, Abo Toru, Kurimoto Miwa, Matsuoka Hiroshi, Matsuno Fumihiko, Nakakuma Hideki

机构信息

Department of Hematology/Oncology, Wakayama Medical University, Kimi-idera 811-1, Wakayama, 641-8510, Japan.

Department of Pathology, Faculty of Medicine, University of Miyazaki, Miyazaki, Japan.

出版信息

Int J Hematol. 2009 Sep;90(2):217-225. doi: 10.1007/s12185-009-0382-6. Epub 2009 Jul 29.

Abstract

We report seven large B cell lymphoma patients showing the involvement of tumor cells with cyclin D3 (CCND3) expression in bone marrow (BM) at the initial diagnosis. All patients presented with B symptoms, splenomegaly, and anemia/thrombocytopenia lacking hemophagocytosis in the BM. Five of the seven patients had suffered from immunological diseases or cancers. The tumor cells were divided into those with a lymphoplasmacytoid or blastoid appearance. Six cases were confirmed to express CD5 antigen on tumor cells. Three cases presented a chromosomal translocation between CCND3 and the immunoglobulin heavy chain (IGH) loci, t(6;14)(p21;q32). Three and two cases showed unmutated and mutated sequences of the variable region of IGH (VH), respectively, and one case showed deletion of an entire segment of VH. Two cases with t(6;14)(p21;q32) showed an unmutated VH sequence and chromosomal translocation within the switch region of IGH. Further studies are required to determine whether CCND3 expression is associated with a unique subset of diffuse large B cell lymphoma.

摘要

我们报告了7例初诊时骨髓(BM)中肿瘤细胞伴有细胞周期蛋白D3(CCND3)表达的大B细胞淋巴瘤患者。所有患者均有B症状、脾肿大,且骨髓中无噬血细胞现象的贫血/血小板减少。7例患者中有5例曾患免疫性疾病或癌症。肿瘤细胞分为淋巴浆细胞样或原始细胞样外观。6例经证实肿瘤细胞表达CD5抗原。3例出现CCND3与免疫球蛋白重链(IGH)基因座之间的染色体易位,即t(6;14)(p21;q32)。3例和2例分别显示IGH可变区(VH)序列未突变和突变,1例显示VH整个片段缺失。2例t(6;14)(p21;q32)患者显示未突变的VH序列以及IGH转换区内的染色体易位。需要进一步研究以确定CCND3表达是否与弥漫性大B细胞淋巴瘤的一个独特亚群相关。

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