Ichinohasama R, Miura I, Kobayashi N, Saitoh Y, DeCoteau J F, Saiki Y, Mori S, Kadin M E, Ooya K
Department of Oral Pathology, Tohoku University School of Dentistry, Sendai, Japan.
Am J Surg Pathol. 1998 Dec;22(12):1528-37. doi: 10.1097/00000478-199812000-00010.
At present, there is no case report of HHV8- primary effusion lymphoma (PEL) with t(9;14)(p13;q32) involving both PAX-5 and immunoglobulin heavy chain gene rearrangement, which is a rare translocation in B-cell non-Hodgkin's lymphoma, in an HIV- patient. We examined an HIV-seronegative 63-year-old Japanese man with hepatitis C virus-associated liver cirrhosis and hepatocellular carcinoma manifesting peritoneal lymphomatous effusion without tumor mass at any body site. The lymphoma cells were examined twice by light microscopy, immunohistochemistry, three-color flow cytometry, cytogenetics, and molecular analyses. The nuclear morphology of lymphoma cells was similar to that of large noncleaved cells, although the lymphoma cell size was a little smaller that of the usual large-cell lymphoma. Immunophenotyping of lymphoma cells in the ascitic fluid revealed a mature peripheral B-cell phenotype (CD5- CD10- CD19+ CD20+ CD22+ Ig G+ lambda+). Cytogenetics showed a clonal population: 45,X,-Y, der(2) t(2;6)(q31;p21.3), t(4;8)(q21;q11.2), der(6) t(2;6)(q31;p21.3) add(6)(q15), t(9;14)(p13;q32.3) [10]/47, idem, +der(6) t(2;6), +16[10]. Southern blot analysis revealed rearranged fragments with a probe for immunoglobulin heavy chain, some of which were a size similar to those with a PAX-5 gene probe. Polymorphism, not rearrangement, of the c-MYC gene, was also found. HHV8 and the Epstein-Barr virus were not detected by polymerase chain reaction. This case is the first report of an HHV8- PEL with t(9;14) involving a PAX-5 gene rearrangement in an HIV-seronegative patient. This primary effusion lymphoma manifested spontaneous regression without any therapy. These findings suggest that there may be an additional subcategory of primary effusion lymphoma that is not associated with HHV8 nor c-MYC(R) but is pathogenetically associated with the PAX-5 gene or hepatitis C virus.
目前，在HIV阴性患者中，尚无HHV8相关性原发性渗出性淋巴瘤（PEL）伴t(9;14)(p13;q32)累及PAX-5和免疫球蛋白重链基因重排的病例报告，这种情况在B细胞非霍奇金淋巴瘤中是一种罕见的易位。我们检查了一名63岁的HIV血清阴性日本男性，他患有丙型肝炎病毒相关性肝硬化和肝细胞癌，表现为腹膜淋巴瘤性渗出，身体任何部位均无肿瘤肿块。对淋巴瘤细胞进行了两次光镜、免疫组化、三色流式细胞术、细胞遗传学和分子分析。淋巴瘤细胞的核形态与大无裂细胞相似，尽管淋巴瘤细胞大小比通常的大细胞淋巴瘤略小。腹水淋巴瘤细胞的免疫表型显示为成熟外周B细胞表型（CD5-CD10-CD19+CD20+CD22+Ig G+lambda+）。细胞遗传学显示一个克隆群体：45,X,-Y, der(2) t(2;6)(q31;p21.3), t(4;8)(q21;q11.2), der(6) t(2;6)(q31;p21.3) add(6)(q15), t(9;14)(p13;q32.3) [10]/47, 同前, +der(6) t(2;6), +16[10]。Southern印迹分析显示用免疫球蛋白重链探针检测到重排片段，其中一些片段大小与用PAX-5基因探针检测到的片段相似。还发现了c-MYC基因的多态性而非重排。聚合酶链反应未检测到HHV8和EB病毒。该病例是首例HIV血清阴性患者中伴t(9;14)累及PAX-5基因重排的HHV8阴性PEL报告。这种原发性渗出性淋巴瘤未经任何治疗即出现自发消退。这些发现提示可能存在原发性渗出性淋巴瘤的一个额外亚类，其与HHV8和c-MYC(R)均无关，但在发病机制上与PAX-5基因或丙型肝炎病毒相关。
