Su I J, Tsai T F, Cheng A L, Chen C C
Department of Pathology, National Taiwan University Hospital and College of Medicine, Taipei.
J Am Acad Dermatol. 1993 Nov;29(5 Pt 1):685-92. doi: 10.1016/0190-9622(93)70231-h.
In addition to human T-lymphotropic virus (HTLV-I), the Epstein-Barr virus (EBV) has recently been demonstrated to be associated with cutaneous T-cell lymphoma (CTCL).
Our purpose was to investigate characteristic clinicopathologic features of the cutaneous lesions of EBV-associated T-cell malignancies.
Clinical records, laboratory data, and histopathologic sections were reviewed. Freshly frozen tumor tissues were immunophenotyped. Southern blot and in situ hybridization studies were performed to detect the EBV genomes.
Ten of 35 CTCL biopsy specimens collected between 1985 and 1992 were found to be EBV-associated. Clonotypic proliferation of EBV genomes was demonstrated in each case, and the atypical T lymphoid cells contained EBV genomes. The cutaneous eruptions of these patients included multiple violaceous papules or nodules, chronic ulcers, and tumors on the trunk or extremities. Three distinct clinicopathologic subgroups could be recognized. The most consistent was the angiocentric T-cell lymphoma or lymphomatoid granulomatosis (type III CTCL) (four cases), presenting with chronic ulcers or violaceous papules. The second group was the T large-cell lymphoma (type II CTCL), Ki-1 antigen (CD30) (positive or negative) (four cases). Three patients with Ki-1- lymphoma had fulminant disease, whereas the remaining Ki-1+ case had a benign course. The third group was the secondary type CTCL (type V CTCL) (two cases), representing systemic EBV-associated T-cell lymphoma. The prognosis was grave. The common features of these EBV-associated CTCLs are resistance to conventional chemotherapy, poor prognosis, and the terminal manifestation of a hemophagocytic syndrome. No EBV genome could be detected in 12 cases of classic CTCL/mycosis fungoides (type I CTCL), or in three cases of HTLV-I-associated adult T-cell lymphoma (type IV CTCL).
Three distinct clinicopathologic subtypes of EBV-associated CTCL were recognized, including one additional type of virus-associated CTCL.
除人类嗜T淋巴细胞病毒（HTLV-I）外，最近发现爱泼斯坦-巴尔病毒（EBV）与皮肤T细胞淋巴瘤（CTCL）有关。
我们的目的是研究EBV相关T细胞恶性肿瘤皮肤病变的特征性临床病理特征。
回顾临床记录、实验室数据和组织病理学切片。对新鲜冷冻的肿瘤组织进行免疫表型分析。进行Southern印迹和原位杂交研究以检测EBV基因组。
在1985年至1992年间收集的35例CTCL活检标本中，有10例被发现与EBV有关。在每个病例中均证实了EBV基因组的克隆性增殖，并且非典型T淋巴细胞含有EBV基因组。这些患者的皮肤疹包括多个紫红色丘疹或结节、慢性溃疡以及躯干或四肢的肿瘤。可识别出三个不同的临床病理亚组。最常见的是血管中心性T细胞淋巴瘤或淋巴瘤样肉芽肿（III型CTCL）（4例），表现为慢性溃疡或紫红色丘疹。第二组是T大细胞淋巴瘤（II型CTCL），Ki-1抗原（CD30）（阳性或阴性）（4例）。3例Ki-1淋巴瘤患者病情凶险，而其余Ki-1阳性病例病程良性。第三组是继发性CTCL（V型CTCL）（2例），代表系统性EBV相关T细胞淋巴瘤。预后严重。这些EBV相关CTCL的共同特征是对传统化疗耐药、预后不良以及噬血细胞综合征的终末期表现。在12例经典CTCL/蕈样肉芽肿（I型CTCL）或3例HTLV-I相关成人T细胞淋巴瘤（IV型CTCL）中未检测到EBV基因组。
识别出了EBV相关CTCL的三种不同临床病理亚型，包括一种额外的病毒相关CTCL类型。
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