Human T cell lymphotropic virus type I and cutaneous T cell leukemia/lymphoma.
Human T cell lymphotropic virus type I and cutaneous T cell leukemia/lymphoma.
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DOI：
10.1084/jem.180.5.1581
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发表时间：
1994-11-01
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影响因子：
--
通讯作者：
Hall WW
中科院分区：
文献类型：
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作者：
Hall WW
T淋巴细胞与罕见临床疾病的关联（1,2）。HTLV-I感染在许多地理区域流行，包括日本、加勒比、南美洲和非洲的部分地区。尽管在流行地区的感染率非常高，多达30%的人口可能被感染，但相对较少的感染者发病，据估计，发生htlv - 1相关临床疾病的终生风险低于5%（1,3,4）。绝大多数感染者仍然是无症状携带者，并成为病毒进一步传播的来源。传播主要有三种途径：母婴垂直传播，主要通过母乳喂养；异性恋和同性恋传播；并通过受污染的血液制品，这可能发生在输血后或静脉药物滥用（1,2）。在已确定的流行地区之外，感染率尚不清楚，但似乎相对较低。在美国，对随机选择的献血者的研究表明，这一比例可能在0.0016%左右(2)，欧洲可能也存在类似的比例。在非流行地区，传播方式可能与流行地区相同。然而，在那些血液供应没有常规筛查HTLV-I感染的国家，输血似乎非常重要。在流行地区，HTLV-I感染与许多不同的临床疾病有关。这些包括成人T细胞白血病（ATL），一种CD4+淋巴细胞的恶性肿瘤，一种皮肤T细胞白血病/淋巴瘤（CTCL）（5,6），一种被称为热带痉挛性截瘫和htlv - 1相关脊髓病（TSP/HAM）的慢性脑脊髓病（7,8），以及一种特征性葡萄膜炎，htlv - 1相关葡萄膜炎（HUV）（9,10）。此外，有研究表明htlv - 1可能与其他炎症过程有关，包括T细胞肺泡炎（11）、多发性肌炎（12）、关节炎（13,14）、感染性皮炎（15）和干燥综合征（16）。然而，目前尚不清楚感染与后一组疾病的关联是否仅仅是巧合，需要进一步的研究来明确确定病毒在其发病机制中的作用。最近，有证据表明，在某些非流行地区，htlv - 1可能与ATL以外的许多ctcl相关。这一观点得到了Manca等人（17）在本期《实验医学杂志》（The Journal of Experimental Medicine）上发表的一篇文章的有力支持，该文章采用PCR检测了蕈样真菌病患者HTLV-I感染。在这篇评论中，htlv - 1在ctcl病因学中的作用的证据将被回顾和总结。ATL于20世纪70年代在日本首次被认为是一种独特的临床疾病（18），是一种成熟CD4§T淋巴细胞的恶性肿瘤，以皮肤受累为特征。这种疾病被分为四种类型：所谓的急性、慢性、阴燃型和淋巴瘤型（10）。该疾病的急性形式是一种极具侵略性的疾病，其特征是高级别白血病，皮肤病变，以及由于白血病细胞浸润到肝、脾、肺、淋巴结和骨髓而引起的广泛的全身累及。此外，溶解性骨病变常伴有高钙血症。皮肤受累的特点是白血病细胞主要浸润到真皮和皮下组织。虽然表皮浸润也会发生，但这种情况相对较少。HTLV-I原病毒单克隆整合在白血病细胞中，大多数患者具有高…
T lymphocytes and an association with rare clinical disorders (1, 2). HTLV-I infection is endemic in a number of geographic regions which include parts of Japan, the Caribbean, South America, and Africa. Despite very high rates of infection in endemic areas where as many as 30% of the population may be infected, relatively few infected individuals develop disease, and it has been estimated that the lifetime risk of developing a HTLV-I-related clinical disorder is less than 5%(1, 3, 4). The vast majority of infected individuals remain as asymptomatic carriers, and serve as a source of further transmission of the virus. Transmission occurs by three major routes: vertically from mother to child, which occurs primarily through breast-feeding; heterosexual and homosexual transmission; and via contaminated blood products, which may occur after blood transfusion or by intravenous drug abuse (1, 2). Outside of established endemic areas, the rates of infection are unknown, but appear to be comparatively low. In the United States, studies on randomly chosen blood donors have indicated that this may be in the region of 0.0016%(2), and similar rates probably exist in Europe. In nonendemic areas, the modes of transmission are presumably the same as in endemic regions. However, it seems likely that blood transfusion is very important in those countries where the blood supply is not routinely screened for HTLV-I infection. In endemic areas, HTLV-I infection is associated with a number of diverse clinical disorders. These include adult T cell leukemia (ATL), a malignancy of CD4+ lymphocytes, and a form of cutaneous T cell leukemia/lymphoma (CTCL)(5, 6), a chronic encephalomyelopathy known both as tropical spastic paraparesis and HTLV-I-associated myelopathy (TSP/HAM)(7, 8), and a characteristic uveitis, HTLV-I-associated uveitis (HUV)(9, 10). In addition, there have been suggestions that HTLV-I may be associated with other inflammatory processes including T cell alveolitis (11), polymyositis (12), arthritis (13, 14), infective dermatitis (15), and Sjogrens syndrome (16). However, it is unclear if the association of infection with the latter group of disorders is merely coincidental, and further studies will be required to definitely establish a role of the virus in their pathogenesis. Very recently, there have been suggestions that in certain nonendemic areas, HTLV-I may be associated with a number of CTCLs other than ATL. This view is strongly supported in a publication by Manca et al.(17) in this issue of The Journal of Experimental Medicine where the PCR was employed to detect HTLV-I infection in patients with mycosis fungoides. In this commentary, the evidence for a role of HTLV-I in the etiology of CTCLs will be reviewed and summarized. ATL, which was first recognized as a unique dinical disorder in Japan in the 1970s (18), is a malignancy of mature CD4 § T lymphocytes with characteristic cutaneous involvement. The disorder has been classified into four types: the so-called acute, chronic, smouldering, and lymphoma types (10). The acute form of the disease is an extremely aggressive disorder characterized by a high-grade leukemia, skin lesions, and widespread systemic involvement resulting from infiltration of leukemic cells in liver, spleen, lungs, lymph nodes, and bone marrow. In addition, lytic bone lesions with an associated hypercalcemia commonly occurs. Cutaneous involvement is characterized by infiltration of leukemic cells primarily into the dermis and subcutaneous tissues. While epidermal infiltration also occurs, this is comparatively rare. The HTLV-I provirus is monoclonally integrated in the leukemic cells, and the majority of patients have high …