Huhn D, Rodt H, Thiel E, Grosse-Wilde H, Fink U, Theml H, Jäger G, Steidle C, Thierfelder S
Blut. 1976 Sep;33(3):141-60. doi: 10.1007/BF00995996.
9 adult patients suffering from different forms of T-cell-malignancies were investigated: 4 patients with T-ALL; 1-T-ALL-CLL mixed form (prolymphocytic); 2 T-CLL; 2 Sézary-syndrome. The clinical peculiarities of the different forms of leukemias were compared: involvement of lymph nodes and spleen, of the central nervous system and the skin was frequent; in contrast to the findings in Sézary-syndrome, bone marrow infiltration was prominent. Light and electron microscopic morphology of the malignant cells are described. In all cases a strong activity of acid phosphatase was demonstrated, in one patient prominent deposits of glycogen. The T-cell-quality of the respective malignant cell population as well as the B-T-cell distribution of the remaining "normal" lymphocytes were shown by the following cell markers: demonstration of T-cell-antigen, resp. membrane immunoglobulins with the aid of specific heterologous antisera conjugated with peroxidase, 125iodine or fluoresceine; complement consumtion or cytotoxicity with such antisera; spontaneous rosette formation with sheep red cells or with acrylic acid beads. Usually, there was a good coincidence in results obtained with the different markers. In two patients, however, T-cells demonstrated by anti-T-globulin were not able to form T-rosettes. Responsiveness of the malignant T-cells and also of the remaining "normal" blood lymphocytes to different mitogens usually was depressed, immunoglobulin levels in the blood mostly were normal. Taking all findings into consideration, T-cell-leukemias of the adult represent a special group of hematological malignancies; the different subgroups show similarities; transitional forms occur.
对9例患有不同形式T细胞恶性肿瘤的成年患者进行了研究:4例T细胞急性淋巴细胞白血病(T-ALL)患者;1例T-ALL-CLL混合形式(幼淋巴细胞性);2例T细胞慢性淋巴细胞白血病(T-CLL);2例 Sézary综合征。比较了不同形式白血病的临床特点:淋巴结、脾脏、中枢神经系统和皮肤受累较为常见;与Sézary综合征的表现不同,骨髓浸润较为突出。描述了恶性细胞的光镜和电镜形态。在所有病例中均显示酸性磷酸酶活性较强,1例患者糖原沉积明显。通过以下细胞标志物显示了各恶性细胞群体的T细胞性质以及其余“正常”淋巴细胞的B-T细胞分布:T细胞抗原的显示,即借助与过氧化物酶、125碘或荧光素偶联的特异性异种抗血清检测膜免疫球蛋白;用此类抗血清进行补体消耗或细胞毒性试验;与绵羊红细胞或丙烯酸珠自发形成玫瑰花结。通常,不同标志物的检测结果吻合良好。然而,在2例患者中,抗T球蛋白显示的T细胞无法形成T玫瑰花结。恶性T细胞以及其余“正常”血液淋巴细胞对不同有丝分裂原的反应性通常降低,血液中的免疫球蛋白水平大多正常。综合所有发现,成人T细胞白血病是血液系统恶性肿瘤中的一个特殊群体;不同亚组表现出相似性;存在过渡形式。