Shimoyama M, Minato K, Tobinai K, Nagai M, Setoya T, Takenaka T, Ishihara K, Watanabe S, Hoshino H, Miwa M, Kinoshita M, Okabe S, Fukushima N, Inada N
Jpn J Clin Oncol. 1983;13 Suppl 2:165-87.
The diverse clinical manifestations of 10 cases of so-called adult T-cell leukemia-lymphoma (ATL)-related T-cell malignancies are described. These cases were anti-ATLA [antibody to ATL virus (ATLV)-associated antigen (ATLA)] positive, and tumor cells had the inducer/helper T-cell phenotype and expressed ATLA when cultured, indicating that these diseases are the same as typical ATL, even though their clinical diagnoses were different from ATL. Accordingly, they are called atypical ATL. Clinically, they could be divided into two subtypes, smoldering type and lymphoma type. In the smoldering type, the disease usually started with skin lesions and rarely with lung lesions. After a prodromal stage of several years, the disease progressed insidiously to the leukemic stage without additional manifestations. The flower cells characteristic of typical ATL were observed in only a small percentage of peripheral lymphoid cells. In two of the five patients the disease progressed to typical ATL after several years from onset. All five patients are alive with a long survival time, more than 6 yr in four, and had high titers of anti-ATLA, suggesting that anti-ATLA might have some role in regulating the proliferation of ATL cells in vivo. In the lymphoma type, morphological diagnosis was not always specific for discriminating ATL-related from ATL-unrelated T-cell lymphomas. Detection of anti-ATLA in the patient's serum and of ATLA in cultured tumor cells, examination of the sera of members of the patient's family for anti-ATLA, and observation of typical flower cells in the peripheral blood though the patients had neither lymphocytosis nor leukemic changes, seem to be useful for the discrimination, especially in an ATL-nonendemic area. Members of the family of a patient with anti-ATLA positive T-lymphoma in an ATL-nonendemic area were also anti-ATLA positive, indicating that healthy ATLV carriers in an ATL-nonendemic area exist as a family colony. This is responsible for sporadic outbreaks of ATL in an ATL-nonendemic area. In summary, the disease entity of ATL is considered, at present, to be a malignancy of inducer/helper T-cells transformed by ATLV or HTLV (human T-cell leukemia virus). In this sense, diverse clinical manifestations of ATL should be recognized as events of viral oncogenesis and host immune response.
本文描述了10例所谓成人T细胞白血病-淋巴瘤（ATL）相关T细胞恶性肿瘤的多样临床表现。这些病例抗ATLA（抗ATL病毒（ATLV）相关抗原（ATLA）抗体）呈阳性，肿瘤细胞具有诱导/辅助T细胞表型，且培养时表达ATLA，这表明这些疾病与典型ATL相同，尽管其临床诊断与ATL不同。因此，它们被称为非典型ATL。临床上，它们可分为两个亚型，即隐匿型和淋巴瘤型。隐匿型中，疾病通常始于皮肤病变，很少始于肺部病变。经过数年的前驱期后，疾病隐匿进展至白血病期，无其他表现。典型ATL特征性的花细胞仅在少数外周淋巴细胞中观察到。5例患者中有2例在发病数年之后疾病进展为典型ATL。所有5例患者均存活，生存时间长，4例超过6年，且抗ATLA滴度高，提示抗ATLA可能在体内调节ATL细胞增殖中发挥一定作用。在淋巴瘤型中，形态学诊断对于区分ATL相关和ATL不相关的T细胞淋巴瘤并不总是具有特异性。检测患者血清中的抗ATLA和培养肿瘤细胞中的ATLA，检查患者家庭成员血清中的抗ATLA，以及尽管患者既无淋巴细胞增多也无白血病改变但观察外周血中的典型花细胞，似乎有助于鉴别，尤其是在非ATL流行地区。在非ATL流行地区，抗ATLA阳性T淋巴瘤患者的家庭成员抗ATLA也呈阳性，这表明非ATL流行地区的健康ATLV携带者以家族群体形式存在。这是导致非ATL流行地区ATL散发的原因。总之，目前认为ATL的疾病实体是由ATLV或HTLV（人类T细胞白血病病毒）转化的诱导/辅助T细胞恶性肿瘤。从这个意义上讲，ATL的多样临床表现应被视为病毒致癌和宿主免疫反应的事件。
