Kirchner M
Folia Haematol Int Mag Klin Morphol Blutforsch. 1979;106(2):182-96.
As long as the aetiology of acute lymphatic leukaemia of children is not known its therapy is based on clinical experience. Among the values of experience those factors will play a part, the evidence of which during the ALL initial stage will be a risk for successful therapy and survival rate. This results in a choice of more aggressive variants of modern therapy schemes. In a cytogenetic study made in 35 children with ALL it was tested, whether even leukaemic chromosome clones will be a risk for the course of acute leukaemia. The duration of the first remission and survival rate were considered as criteria. The evidence of a leukaemic chromosome clone could be shown to be followed by a short survival rate, irrespective of the stage of the disease where the clone had been observed first. Thus, cytostatic therapy in those ALL patients who are affected with luekaemic chromosome aberration of stem line character should be aimed at the complete annihilation of the clone, irrespective of other remission criteria. The failure of blood and bone-marrow cultures as early as during the untreated initial stage indicated a primary cellular immuno-insufficiency. This combination of cell immuno-depression with high peripheral leukocytes connts and a primary mediastinal tumour or a generalizing lymphosarcoma respectively, was the highest risk up till now for the course of the disease. Judging from the duration of the first remission and the survival rates, the consecutive schemes of therapy did not differ in their effect on leukaemia with pathological stem lines. On the basis of the present study the impression could not be excluded that up till now long term survival rates could be attributed rather to individual manners of response than to the modern therapy scheme.
只要儿童急性淋巴细胞白血病的病因不明,其治疗就基于临床经验。在经验值中,那些因素将发挥作用,其在急性淋巴细胞白血病初始阶段的证据将是成功治疗和生存率的一个风险因素。这导致选择更积极的现代治疗方案变体。在对35名急性淋巴细胞白血病儿童进行的细胞遗传学研究中,测试了白血病染色体克隆是否会成为急性白血病病程的一个风险因素。首次缓解期的持续时间和生存率被视为标准。可以证明,白血病染色体克隆的出现会伴随着较短的生存率,而与首次观察到克隆的疾病阶段无关。因此,对于那些患有具有干细胞系特征的白血病染色体畸变的急性淋巴细胞白血病患者,细胞抑制疗法应旨在彻底消灭克隆,而不考虑其他缓解标准。早在未经治疗的初始阶段血液和骨髓培养失败就表明存在原发性细胞免疫功能不全。这种细胞免疫抑制与外周血白细胞计数高以及分别伴有原发性纵隔肿瘤或全身性淋巴肉瘤的组合,是迄今为止该疾病病程的最高风险因素。从首次缓解期的持续时间和生存率来看,连续的治疗方案对具有病理性干细胞系的白血病的疗效并无差异。根据目前的研究,不能排除这样一种印象,即迄今为止长期生存率更多地归因于个体的反应方式而非现代治疗方案。