Waldmann T A, Greene W C, Sarin P S, Saxinger C, Blayney D W, Blattner W A, Goldman C K, Bongiovanni K, Sharrow S, Depper J M
J Clin Invest. 1984 Jun;73(6):1711-8. doi: 10.1172/JCI111379.
Adult T cell leukemia (ATL) and Sézary leukemia are malignant proliferations of T lymphocytes that share similar cell morphology and clinical features. ATL is associated with HTLV (human T cell leukemia/lymphoma virus), a unique human type C retrovirus, whereas most patients with the Sézary syndrome do not have antibodies to this virus. Leukemic cells of both groups were of the T3, T4-positive, T8-negative phenotype. Despite the similar phenotype, HTLV-negative Sézary leukemic cells frequently functioned as helper cells, whereas some HTLV-positive ATL and HTLV-positive Sézary cells appeared to function as suppressors of immunoglobulin synthesis. One can distinguish the HTLV-positive from the HTLV-negative leukemias using a monoclonal antibody (anti-Tac) that appears to identify the human receptor for T cell growth factor (TCGF). Resting normal T cells and most HTLV-negative Sézary cells were Tac-negative, whereas all ATL cell populations were Tac-positive. The observation that ATL cells manifest TCGF receptors suggests the possibility that an abnormality of the TCGF-TCGF receptor system may partially explain the uncontrolled growth of these cells.
成人T细胞白血病(ATL)和蕈样霉菌病白血病是T淋巴细胞的恶性增殖,具有相似的细胞形态和临床特征。ATL与人类T细胞白血病/淋巴瘤病毒(HTLV)有关,HTLV是一种独特的人类C型逆转录病毒,而大多数蕈样霉菌病综合征患者对此病毒没有抗体。两组的白血病细胞均为T3、T4阳性、T8阴性表型。尽管表型相似,但HTLV阴性的蕈样霉菌病白血病细胞常发挥辅助细胞的功能,而一些HTLV阳性的ATL和HTLV阳性的蕈样霉菌病细胞似乎发挥免疫球蛋白合成抑制因子的作用。使用一种单克隆抗体(抗Tac)可以区分HTLV阳性和HTLV阴性白血病,该抗体似乎能识别T细胞生长因子(TCGF)的人类受体。静止的正常T细胞和大多数HTLV阴性的蕈样霉菌病细胞为Tac阴性,而所有ATL细胞群体均为Tac阳性。ATL细胞表现出TCGF受体这一观察结果提示,TCGF-TCGF受体系统异常可能部分解释了这些细胞的失控生长。