Brusamolino E, Bertazzoni U, Isernia P, Ginelli E, Scovassi A I, Zurlo M G, Plevani P, Sacchi N, Bernasconi C
Adv Exp Med Biol. 1982;145:279-303. doi: 10.1007/978-1-4684-8929-3_24.
Terminal Transferase (TdT), Adenosine Deaminase (ADA), immunological membrane markers, cytochemical reactivity and cytogenetics were analyzed in 226 patients with ALL, AUL and AML, in 70 patients with CML and in 3 cases of Ph' positive acute leukemia presenting as ALL. TdT was tested in peripheral blood and bone marrow with both the biochemical and immunofluorescence (IF) methods, and ADA was determined biochemically only in peripheral blood cells. By using conventional cytochemistry, cell surface markers determinations, TdT and ADA analysis, three distinct groups are recognized in ALL at presentation: T-ALL with TdT+ and very high ADA values; non-T, non-B ALL with TdT+ and intermediate levels of ADA; B-ALL with TdT absence and low levels of ADA. Clinical presentation and responses to therapy in adult and children ALL were correlated to TdT determinations. The median survivals in adults, calculated for TdT+ and TdT- groups, were 14.2 and 5.6 months, respectively. TdT and ADA were determined in ALL during remission. The wide fluctuation observed for TdT IF and ADA values prevented a reliable monitoring of remissions. At relapse, TdT and ADA values were similar to those found for ALL at presentation; TdT IF determinations were diagnostic in cases showing CNS involvement as the only localization. Forty per cent of AUL and 11% of AML cases were positive for TdT; the medians of ADA values of the TdT+ cases in both AML and AUL were several times higher than those obtained in the TdT- group. While TdT positivity and high ADA had a favorable prognostic value in AUL, similar conclusions can not be drawn at the moment for AML. In chronic phase of CML, TdT was strictly negative and ADA values were increased over the control line only in cases showing initial signs of transformation. In acute phase, the cases positive for TdT (32%) presented a significantly higher ADA activity than the TdT negative ones. The actuarial survival curves for the TdT+ and TdT- groups differ significantly, presenting median survivals from onset of phase of 11 and 4.8 months respectively. The three cases of Ph' positive ALL were all TdT+, presented high ADA values and entered chronic phase of CML after therapy.
对226例急性淋巴细胞白血病(ALL)、急性未分化白血病(AUL)和急性髓系白血病(AML)患者、70例慢性髓系白血病(CML)患者以及3例表现为ALL的Ph'阳性急性白血病患者进行了末端转移酶(TdT)、腺苷脱氨酶(ADA)、免疫膜标志物、细胞化学反应性和细胞遗传学分析。采用生化法和免疫荧光(IF)法检测外周血和骨髓中的TdT,仅采用生化法测定外周血细胞中的ADA。通过常规细胞化学、细胞表面标志物测定、TdT和ADA分析,在ALL初诊时可识别出三个不同的组:TdT阳性且ADA值极高的T-ALL;TdT阳性且ADA水平中等的非T、非B-ALL;TdT阴性且ADA水平低的B-ALL。成人和儿童ALL的临床表现及对治疗的反应与TdT测定结果相关。TdT阳性和阴性组成人的中位生存期分别为14.2个月和5.6个月。在ALL缓解期测定了TdT和ADA。TdT IF和ADA值的广泛波动使得无法可靠地监测缓解情况。复发时,TdT和ADA值与ALL初诊时相似;TdT IF测定对仅表现为中枢神经系统受累的病例具有诊断价值。40%的AUL病例和11%的AML病例TdT呈阳性;AML和AUL中TdT阳性病例的ADA值中位数比TdT阴性组高几倍。虽然TdT阳性和高ADA在AUL中具有良好的预后价值,但目前在AML中尚不能得出类似结论。在CML慢性期,TdT严格阴性,仅在显示初始转化迹象的病例中ADA值高于对照线。在急性期,TdT阳性的病例(32%)的ADA活性明显高于TdT阴性的病例。TdT阳性和阴性组的精算生存曲线差异显著,从阶段开始的中位生存期分别为11个月和4.8个月。3例Ph'阳性ALL病例均为TdT阳性,ADA值高,治疗后进入CML慢性期。