Crist W, Pullen J, Boyett J, Falletta J, van Eys J, Borowitz M, Jackson J, Dowell B, Russell C, Quddus F
St. Jude Children's Research Hospital, Memphis.
J Clin Oncol. 1988 Jan;6(1):34-43. doi: 10.1200/JCO.1988.6.1.34.
Analysis of remission induction rates for 1,768 children (1.5 to 11 years) and 425 adolescents (greater than or equal to 11 years) with acute lymphoid leukemia (ALL), and of event-free survival times for 570 children and 147 adolescents, disclosed that adolescents fared significantly worse by both measures of treatment outcome (P = .0001). Adolescents with either T cell or non-T cell ALL entered remission significantly less often than did children (P = less than .02 and P = less than .001, respectively). Within each of the major immunophenotypes of ALL, adolescents had shorter duration of continuous complete remission: early pre-B (non-B, non pre-B, non-T) (P = .001), pre-B (P = .05), and T (P = .027). We compared the clinical characteristics of adolescents and children, and lymphoblast characteristics present at diagnosis to account for the inferior prognosis of adolescent patients. Adolescents had a higher incidence of T cell ALL (P = .0001) and thus a higher incidence of all T cell-associated characteristics. Adolescents with non-T, non-B ALL were more likely to be male (P = .044), and to have higher leukocyte counts (P = .002) and lower levels of IgG (P = .0003), IgA (P = .0001), and IgM (P = .002). Their leukemic cells had lower PAS scores (P = .0001), a higher incidence rate of L2 morphology by French-American-British (FAB) criteria (P = .001), common ALL antigen negativity (P = .0001), and hypodiploid or pseudodiploid karyotypes (P = .004). These findings clearly indicate an increased incidence of prognostically unfavorable clinical and biologic features in adolescents with ALL, providing a biologic explanation for their poor prognosis.
对1768名1.5至11岁的儿童及425名11岁及以上的青少年急性淋巴细胞白血病(ALL)患者的缓解诱导率,以及570名儿童和147名青少年的无事件生存时间进行分析后发现,青少年在这两项治疗结果指标上均显著较差(P = 0.0001)。患有T细胞或非T细胞ALL的青少年进入缓解期的频率明显低于儿童(分别为P < 0.02和P < 0.001)。在ALL的每种主要免疫表型中,青少年持续完全缓解的持续时间较短:早前B细胞型(非B、非前B、非T)(P = 0.001)、前B细胞型(P = 0.05)和T细胞型(P = 0.027)。我们比较了青少年和儿童的临床特征以及诊断时的淋巴母细胞特征,以解释青少年患者预后较差的原因。青少年T细胞ALL的发病率较高(P = 0.0001),因此所有与T细胞相关特征的发病率也较高。患有非T、非B ALL的青少年更有可能为男性(P = 0.044),白细胞计数较高(P = 0.002),而IgG(P = 0.0003)、IgA(P = 0.0001)和IgM(P = 0.002)水平较低。他们的白血病细胞PAS评分较低(P = 0.0001),按照法国-美国-英国(FAB)标准L2形态的发生率较高(P = 0.001),常见ALL抗原阴性(P = 0.0001),以及亚二倍体或假二倍体核型(P = 0.004)。这些发现清楚地表明,ALL青少年患者中预后不良的临床和生物学特征的发生率增加,为其预后不良提供了生物学解释。