Crist W M, Shuster J J, Falletta J, Pullen D J, Berard C W, Vietti T J, Alvarado C S, Roper M A, Prasthofer E, Grossi C E
Department of Hematology-Oncology, St Jude Children's Research Hospital, Memphis, TN 38101.
Blood. 1988 Dec;72(6):1891-7.
The immunophenotypes of lymphoblasts from children with newly diagnosed T-cell acute lymphoid leukemia (T-ALL, n = 101) or T-cell non-Hodgkin lymphoma (T-NHL, n = 31) were analyzed to correlate stage of thymocyte differentiation with clinical features and outcome. The 67 boys and 34 girls with T-ALL were 1 month to 18 years old (median, 8 years) with leukocyte counts ranging from 2 to 810 x 10(9)/L (median, 55 x 10(9)/L). Eighteen of these patients were black, and 70 had a mediastinal mass. Twenty-six boys and five girls with a median age of 9 years (range, 1 to 20 years) had T-NHL. Seven of these patients were black, and 24 had a mediastinal mass. The distributions of thymocyte developmental stages (early [CD7+], intermediate [CD1+ and/or CD4+ and/or CD8+], and mature [CD3+]) in cases of T-ALL and T-NHL were significantly different: 34%, 43%, and 23% v 6%, 62%, and 32% (P = .02). A comparison of the patients' clinical features according to the maturational stage of thymocytes failed to disclose significant differences in the majority of characteristics studied. However, patients with mature-stage T-NHL, with or without the addition of subjects with mature-stage T-ALL, were less likely to have a mediastinal mass (P = .02 for both comparisons). Those with intermediate-stage T-cell malignancy (T-ALL and T-NHL combined) were the subgroup most likely to have a mediastinal mass (P = .01). Response to remission induction therapy was significantly worse in the T-ALL subgroup with an early-stage phenotype: a failure rate of 21% v 0% and 6% for the two more differentiated phenotypic subgroups (P = .007). Event-free survival was not affected by thymocyte maturational stage in cases of either T-ALL or T-NHL. Despite evidence of clinical heterogeneity among the maturational stages of T-cell malignancies in children, these developmental subdivisions do not appear to be critical determinants of outcome once remission is achieved. We conclude that such phenotypes need not be included in the stratification plans for clinical trials using common induction treatment.
分析新诊断的T细胞急性淋巴细胞白血病（T-ALL，n = 101）或T细胞非霍奇金淋巴瘤（T-NHL，n = 31）患儿淋巴母细胞的免疫表型，以将胸腺细胞分化阶段与临床特征及预后相关联。67名患T-ALL的男孩和34名患T-ALL的女孩年龄在1个月至18岁之间（中位数为8岁），白细胞计数范围为2至810×10⁹/L（中位数为55×10⁹/L）。这些患者中有18名是黑人，70名有纵隔肿块。26名男孩和5名女孩患T-NHL，年龄中位数为9岁（范围为1至20岁）。这些患者中有7名是黑人，24名有纵隔肿块。T-ALL和T-NHL病例中胸腺细胞发育阶段（早期[CD7⁺]、中期[CD1⁺和/或CD4⁺和/或CD8⁺]和成熟[CD3⁺]）的分布有显著差异：34%、43%和23%对比6%、62%和32%（P = 0.02）。根据胸腺细胞成熟阶段对患者临床特征进行比较，在所研究的大多数特征中未发现显著差异。然而，成熟阶段T-NHL的患者，无论是否加入成熟阶段T-ALL的患者，有纵隔肿块的可能性较小（两次比较P均 = 0.02）。中期T细胞恶性肿瘤（T-ALL和T-NHL合并）的患者是最有可能有纵隔肿块的亚组（P = 0.01）。具有早期表型的T-ALL亚组对缓解诱导治疗的反应明显更差：失败率为21%，而另外两个分化程度更高的表型亚组为0%和6%（P = 0.007）。在T-ALL或T-NHL病例中，无事件生存期不受胸腺细胞成熟阶段的影响。尽管有证据表明儿童T细胞恶性肿瘤成熟阶段存在临床异质性，但一旦实现缓解，这些发育细分似乎并不是预后的关键决定因素。我们得出结论，在使用常见诱导治疗的临床试验分层计划中无需纳入此类表型。
