Huang Qin, Wilczynski Sharon P, Chang Karen L, Weiss Lawrence M
Division of Pathology, City of Hope National Medical Center, Duarte, CA 91010, USA.
Am J Clin Pathol. 2006 Aug;126(2):222-9. doi: 10.1309/DDGL-WRV3-KR91-64G7.
We describe a composite lymphoma with recurrent Hodgkin lymphoma and diffuse large B-cell lymphoma components manifesting as a single, perforated small intestinal tumor in a 56-year-old man with a history of classical Hodgkin lymphoma and recent relapse in the bone marrow. The resected mass had 2 morphologically and immunophenotypically distinct components; 1 showed a pleomorphic cellular infiltrate with fibrosis and contained numerous, large Hodgkin/Reed-Sternberg-like cells and variants. The tumor cells were CD30+ and focally positive for CD15 but CD20-, CD79a-, and PAX-5-. In situ hybridization for Epstein-Barr virus (EBV) was strongly positive in the large pleomorphic tumor cells. The adjacent component displayed sheets of relatively uniform, large lymphoid cells with typical morphologic features of diffuse large cell lymphoma. The tumor cells showed uniform expression of tested B-cell antigens, absence of CD30 or CD15, and complete absence of EBV-encoded RNA. Separate molecular studies with immunoglobulin heavy and k light chain gene rearrangements clearly demonstrated an identical rearrangement pattern, indicating derivation from the same clone, which was confirmed by direct DNA sequencing analysis. Such distinctly different morphology, immunophenotype, and EBV status in different components within a clonally related single tumor mass is striking.
我们描述了一例复合性淋巴瘤，其具有复发性霍奇金淋巴瘤和弥漫性大B细胞淋巴瘤成分，表现为一名56岁男性的单个穿孔性小肠肿瘤，该患者有经典霍奇金淋巴瘤病史且近期骨髓复发。切除的肿块有2个形态学和免疫表型不同的成分；1个显示多形性细胞浸润伴纤维化，包含大量大的霍奇金/里德-斯特恩伯格样细胞及变异型。肿瘤细胞CD30阳性，局灶性CD15阳性，但CD20、CD79a和PAX-5阴性。爱泼斯坦-巴尔病毒（EBV）原位杂交在大的多形性肿瘤细胞中呈强阳性。相邻成分显示相对均一的大淋巴细胞片层，具有弥漫性大细胞淋巴瘤的典型形态学特征。肿瘤细胞显示所检测的B细胞抗原均匀表达，无CD30或CD15表达，且完全无EBV编码RNA。通过免疫球蛋白重链和κ轻链基因重排进行的单独分子研究清楚地显示出相同的重排模式，表明源自同一克隆，这通过直接DNA测序分析得以证实。在克隆相关的单个肿瘤块内不同成分中如此明显不同的形态学、免疫表型和EBV状态令人瞩目。
