Hsiao C H, Su I J, Hsieh S W, Huang S F, Tsai T F, Chen M Y, How S W
Department of Pathology, National Taiwan University Hospital, Taipei.
Am J Surg Pathol. 1999 Apr;23(4):482-7. doi: 10.1097/00000478-199904000-00015.
Intravascular lymphomatosis (IL) is an unusual neoplasm characterized by multifocal proliferation of lymphoma cells exclusively within the blood vessels. We report here a patient with acquired immunodeficiency syndrome (AIDS) and disseminated Kaposi's sarcoma. A 233-bp amplification product of HHV-8 was detected in the DNA extracted from specimens of Kaposi's sarcoma at different sites by polymerase chain reaction (PCR). At autopsy, the vessels within the Kaposi's sarcoma were dilated and filled with atypical large mononuclear cells. No such feature was seen in the vessels of non-Kaposi's sarcomatous regions. Immunohistochemically, the spindle cells of Kaposi's sarcoma were positive for CD31 (endothelial cell marker). The intravascular tumor cells were positive for CD45 (leukocyte common antigen) but negative for others, including chloroacetate esterase, CD45-RO (UCHL-1, Pan-T), CD3, CD43, CD20 (L26, Pan-B), CD30 (Ki-1), immunoglobulin heavy chains and light chains, CD56 (natural killer cell antigen), and CD31. Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in the DNA extracts from fresh tissue of Kaposi's sarcoma by PCR, which indicated that the lymphoma cells within the Kaposi's sarcoma were of monoclonal B cell origin. In situ hybridization revealed that EBER-1 transcripts were present in the lymphoma cells of IL but not in the spindle cells of Kaposi's sarcoma. To the authors' best knowledge, this is the first instance of IL in an AIDS patient with direct evidence of EBV association.
血管内淋巴瘤（IL）是一种罕见的肿瘤，其特征是淋巴瘤细胞仅在血管内呈多灶性增殖。我们在此报告一名患有获得性免疫缺陷综合征（AIDS）和播散性卡波西肉瘤的患者。通过聚合酶链反应（PCR）在从不同部位的卡波西肉瘤标本中提取的DNA中检测到HHV-8的233-bp扩增产物。尸检时，卡波西肉瘤内的血管扩张，充满非典型大单核细胞。在非卡波西肉瘤区域的血管中未见此特征。免疫组织化学检查显示，卡波西肉瘤的梭形细胞CD31（内皮细胞标志物）呈阳性。血管内肿瘤细胞CD45（白细胞共同抗原）呈阳性，但对其他标志物呈阴性，包括氯乙酸酯酶、CD45-RO（UCHL-1，全T）、CD3、CD43、CD20（L26，全B）、CD30（Ki-1）、免疫球蛋白重链和轻链、CD56（自然杀伤细胞抗原）和CD31。通过PCR在卡波西肉瘤新鲜组织的DNA提取物中检测到免疫球蛋白重链基因的单克隆重排，这表明卡波西肉瘤内的淋巴瘤细胞起源于单克隆B细胞。原位杂交显示，EBER-1转录本存在于IL的淋巴瘤细胞中，但不存在于卡波西肉瘤的梭形细胞中。据作者所知，这是首例有EBV关联直接证据的AIDS患者发生IL的病例。
