Plüss H J
University Children's Hospital, Zurich, Switzerland.
Helv Paediatr Acta. 1987 Oct;42(2-3):197-247.
The analysis of clinical and hematological data for prognostic relevance in 200 children with acute lymphocytic leukemia (ALL), diagnosed between January 1964 and December 1980, showed that the importance of single risk factors has changed due to improvements in therapy. Morphology and cytochemistry lost their prognostic value they had in those patients treated before October 1971. In the children treated in the seventies, the WBC became the most important prognostic factor, followed by infiltrate size and age. (Age was less important than infiltrates for remission duration, but more for survival.) Immunological markers were evaluated in 56 children, since 1974. Because of the small number, no significance as risk factor was found. Those 25% with E+ blasts tended to have only a slightly worse course than "non-T-non-B"-ALL. Treatment became a highly significant risk factor, because of the improvement in results between those patients treated with CALGB protocol 6801 and those on protocol 7111. Two steps were responsible for this: better treatment strategies, and, most important, CNS-prophylaxis (or "sanctuary"-treatment) in all patients. Even in the sixties, where IT methotrexate alone was given sporadically, omitting the CNS-prophylaxis represented an important risk factor. Since 1971, most patients received cranial irradiation or intermediate dose methotrexate as second mode of CNS-prophylaxis. This resulted in a significant decrease in the incidence of CNS relapse. CNS-prophylaxis mode therefore represented a significant prognostic factor, although age, WBC and infiltrates had become more important. Evaluation of the clinical and hematological data gave the following limits for an increased or lesser risk: WBC over 30.G/l: high risk, under 10.G/l: favourable. Age: below 1 year and over 10 years: high risk, 1-2 years: probably moderately increased risk. Infiltrates: no palpable hepatosplenomegaly and no lymph nodes: favourable, all palpable infiltrates: "standard" or increased risk. Platelets (under 30.G/l) represented a minor good risk factor. The common ALL antigen (CALLA) was not yet examined in this series, calling it a favourable factor is based on recent experience from other centers. T-markers are probably not a risk factor by themselves, but other poor prognostic signs are usually associated and of primary importance. If treatment will be based on risk classification, it is important to keep in mind that treatment improvements might change the significance of any prognostic factor completely.
对1964年1月至1980年12月期间确诊的200例急性淋巴细胞白血病(ALL)患儿的临床和血液学数据进行预后相关性分析,结果显示,由于治疗方法的改进,单一危险因素的重要性已经发生了变化。形态学和细胞化学在1971年10月之前接受治疗的患者中所具有的预后价值已经丧失。在70年代接受治疗的儿童中,白细胞成为最重要的预后因素,其次是浸润灶大小和年龄。(年龄对缓解期的重要性低于浸润灶,但对生存率的影响更大。)自1974年起,对56例儿童进行了免疫标志物评估。由于数量较少,未发现其作为危险因素具有统计学意义。那些有E+母细胞的25%患儿的病程往往仅比“非T非B”型ALL略差。治疗成为一个极具统计学意义的危险因素,这是因为采用CALGB方案6801治疗的患者与采用方案7111治疗的患者相比,治疗效果有所改善。这有两个原因:更好的治疗策略,以及最重要的是,对所有患者进行中枢神经系统预防(或“庇护所”治疗)。即使在60年代,仅偶尔给予鞘内甲氨蝶呤,省略中枢神经系统预防也是一个重要的危险因素。自1971年以来,大多数患者接受了颅脑照射或中等剂量甲氨蝶呤作为中枢神经系统预防的第二种方式。这导致中枢神经系统复发的发生率显著降低。因此,中枢神经系统预防方式是一个重要的预后因素,尽管年龄、白细胞和浸润灶变得更为重要。对临床和血液学数据的评估给出了以下风险增加或降低的界限:白细胞超过30×10⁹/L:高风险,低于10×10⁹/L:预后良好。年龄:1岁以下和10岁以上:高风险,1 - 2岁:可能中度增加风险。浸润灶:无可触及的肝脾肿大和无淋巴结:预后良好,所有可触及的浸润灶:“标准”或增加风险。血小板(低于30×10⁹/L)是一个次要的良好风险因素。本系列中尚未检测普通ALL抗原(CALLA),将其称为有利因素是基于其他中心的近期经验。T标志物本身可能不是一个危险因素,但通常与其他不良预后征象相关且具有首要重要性。如果治疗将基于风险分类,重要的是要记住,治疗方法的改进可能会完全改变任何预后因素的意义。