Greaves M F, Janossy G, Peto J, Kay H
Br J Haematol. 1981 Jun;48(2):179-97.
Leukaemic cells from 542 patients under 21 years of age with a diagnosis of acute lymphoblastic leukaemia (ALL) were typed with immunological cell surface markers between June 1975 and December 1979; 379 of these patients entered into the trials up until December 1978 have been followed for more than 1 year. They were divided into four subgroups: common (c) ALL, T (thymic) ALL, 'null' (or 'unclassified') ALL and a rare lymphoma/leukaemia type B-ALL. A T-cell phenotype was found more frequently in boys and was usually but not invariably associated with a high white cell count at presentation. A mediastinal thymic mass was present in 53% of T-ALL patients but was not observed in any unequivocal not-T ALL. Clinical prognosis differed substantially between the three major phenotypic classes, remission induction rate and remission duration being lowest in T-ALL, better in 'null' ALL, and highest in cALL (P trend less than 0 . 0001; P = 0 . 0002 for comparison of cALL versus T-ALL). There was a much higher incidence of CNS involvement in the T-ALL group than in the cALL group or 'null' All group and although this was strongly correlated with WBC count it was also significantly associated with T-ALL independent of WBC count. Overall in this series and also within the major cALL subclass there is a strong correlation between high WBC count and poor clinical response (remission induction and duration). When the three major immunological subclasses are adjusted for WBC count the prognostic correlation of antigenic phenotype is reduced and statistically insignificant. It is suggested that immunological (and enzymatic) phenotype of ALL subclasses may not be an independent correlate of prognosis but nevertheless is linked to other cell differentiation features, especially growth rate and sites of clonal expansion (e.g. marrow versus thymus), which critically influence the size of the clonogenic leukaemic population and its associated evolutionary status with the respect to drug resistant mutants at the time of diagnosis and introduction of therapy.
1975年6月至1979年12月期间，对542名21岁以下诊断为急性淋巴细胞白血病（ALL）的患者的白血病细胞进行了免疫细胞表面标志物分型；截至1978年12月，这些患者中有379人进入试验并已随访1年以上。他们被分为四个亚组：普通（c）ALL、T（胸腺）ALL、“无”（或“未分类”）ALL和一种罕见的淋巴瘤/白血病B-ALL型。T细胞表型在男孩中更常见，通常但并非总是与就诊时的高白细胞计数相关。53%的T-ALL患者有纵隔胸腺肿块，而在任何明确的非T-ALL患者中均未观察到。三种主要表型类别的临床预后有很大差异，T-ALL的缓解诱导率和缓解持续时间最低，“无”ALL较好，cALL最高（P趋势小于0.0001；cALL与T-ALL比较，P = 0.0002）。T-ALL组中枢神经系统受累的发生率远高于cALL组或“无”ALL组，虽然这与白细胞计数密切相关，但与T-ALL也显著相关，且独立于白细胞计数。总体而言，在本系列以及主要的cALL亚类中，高白细胞计数与不良临床反应（缓解诱导和持续时间）之间存在很强的相关性。当对三种主要免疫亚类进行白细胞计数校正后，抗原表型的预后相关性降低且无统计学意义。提示ALL亚类的免疫（和酶）表型可能不是预后的独立相关因素，但仍与其他细胞分化特征相关，尤其是生长速率和克隆扩增部位（如骨髓与胸腺），这些特征在诊断和开始治疗时对克隆性白血病细胞群体的大小及其与耐药突变体相关的进化状态有至关重要的影响。
