Nador R G, Cesarman E, Chadburn A, Dawson D B, Ansari M Q, Sald J, Knowles D M
Department of Pathology, New York Hospital-Cornell Medical Center, New York 10021, USA.
Blood. 1996 Jul 15;88(2):645-56.
We recently discovered the Kaposi's sarcoma-associated herpes virus (KSHV/HHV-8) in an uncommon and unusual subset of AIDS-related lymphomas that grow mainly in the body cavities as lymphomatous effusions without an identifiable contiguous tumor mass. The consistent presence of KSHV and certain other distinctive features of these body cavity-based lymphomas suggest that they represent a distinct entity. We tested this hypothesis by investigating 19 malignant lymphomatous effusions occurring in the absence of a contiguous tumor mass for their clinical, morphologic, immunophenotypic, viral, and molecular characteristics, KSHV was present in 15 of 19 lymphomas. All four KSHV-negative lymphomatous effusions exhibited Burkitt or Burkitt-like morphology and c-myc gene rearrangements and, therefore, appeared to be Burkitt-type lymphomas occurring in the body cavities. In contrast, all 15 KSHV-positive lymphomatous effusions exhibited a distinctive morphology bridging large-cell immunoblastic lymphoma and anaplastic large-cell lymphoma, and all 12 cases studied lacked c-myc gene rearrangements. In addition, these lymphomas occurred in men (15/15), frequently but not exclusively in association with HIV infection (13/15), in which homosexuality was a risk factor (13/13), presented initially as a lymphomatous effusion (14/15), remained localized to the body cavity of origin (13/15), expressed CD45 (15/15) and one or more activation-associated antigens (9/10) in the frequent absence of B-cell-associated antigens (11/15), exhibited clonal immunoglobulin gene rearrangements (13/13), contained Epstein-Barr virus (14/15), and lacked bcl-2, bcl-6, ras and p53 gene alterations (13/15). These findings strongly suggest that the KSHV-positive malignant lymphomatous effusions represent a distinct clinicopathologic and biologic entity and should be distinguished from other malignant lymphomas occurring in the body cavities. Therefore, we recommend that these malignant lymphomas be designated primary effusion lymphomas (PEL), rather than body cavity-based lymphomas, since this term describes them more accurately and avoids their confusion with other malignant lymphomas that occur in the body cavities. We further recommend that these PEL be considered for inclusion as a new entity in the Revised European-American Lymphoma Classification.
我们最近在一类罕见且特殊的艾滋病相关淋巴瘤中发现了卡波西肉瘤相关疱疹病毒（KSHV/HHV - 8），这类淋巴瘤主要在体腔内以淋巴瘤性积液的形式生长，没有可识别的连续肿瘤块。KSHV的持续存在以及这些基于体腔的淋巴瘤的某些其他独特特征表明它们代表一种独特的实体。我们通过研究19例无连续肿瘤块的恶性淋巴瘤性积液的临床、形态学、免疫表型、病毒学和分子特征来验证这一假设，19例淋巴瘤中有15例存在KSHV。所有4例KSHV阴性的淋巴瘤性积液均表现出伯基特或伯基特样形态以及c - myc基因重排，因此似乎是发生在体腔的伯基特型淋巴瘤。相比之下，所有15例KSHV阳性的淋巴瘤性积液均表现出一种独特的形态，介于大细胞免疫母细胞淋巴瘤和间变性大细胞淋巴瘤之间，且所研究的12例病例均缺乏c - myc基因重排。此外，这些淋巴瘤发生在男性（15/15），常但并非仅与HIV感染相关（13/15），其中同性恋是一个危险因素（13/13），最初表现为淋巴瘤性积液（14/15），局限于原发体腔（13/15），表达CD4（15/15）以及一种或多种激活相关抗原（9/10），且常缺乏B细胞相关抗原（11/15），表现出克隆性免疫球蛋白基因重排（13/13），含有EB病毒（14/15），并且缺乏bcl - 2、bcl - 6、ras和p53基因改变（13/15）。这些发现强烈表明KSHV阳性的恶性淋巴瘤性积液代表一种独特的临床病理和生物学实体，应与发生在体腔的其他恶性淋巴瘤相区分。因此，我们建议将这些恶性淋巴瘤命名为原发性渗出性淋巴瘤（PEL），而不是基于体腔的淋巴瘤，因为这个术语能更准确地描述它们，避免与发生在体腔的其他恶性淋巴瘤混淆。我们进一步建议将这些PEL考虑纳入修订后的欧美淋巴瘤分类中作为一个新的实体。
