Lilleyman J S, Eden O B
Med Pediatr Oncol. 1986;14(3):182-6. doi: 10.1002/mpo.2950140314.
Four groups of children with poor-prognosis disease were selected for study from the Medical Research Council childhood ALL trials. Each group was reviewed for distinguishing clinical features and response to therapy. The four comprised those with a diagnostic white cell count over 50 X 10(9)/l, those with a mediastinal mass, those less than a year old at diagnosis, and those presenting with meningeal involvement. They accounted for 17, 5.3, 2.6, and 3%, respectively, of unselected children with ALL. As expected, all four categories showed significantly inferior relapse-free survival to the rest, but only in patients with a mediastinal mass and in the under-1-year-olds did a recognisable syndrome emerge clearly associated with other clinical features. These two syndromes, high-count T-ALL and high-count infant null-ALL, are logical candidates for alternative therapy. Established meningeal disease also continues to present a major therapeutic challenge.
从医学研究委员会儿童急性淋巴细胞白血病试验中选取了四组预后不良疾病的儿童进行研究。对每组儿童的特征性临床特点和治疗反应进行了评估。这四组儿童分别为诊断时白细胞计数超过50×10⁹/L者、有纵隔肿块者、诊断时年龄小于1岁者以及有脑膜受累者。他们分别占未经过筛选的急性淋巴细胞白血病儿童的17%、5.3%、2.6%和3%。正如预期的那样,所有这四类儿童的无复发生存率均显著低于其他儿童,但只有纵隔肿块患儿和1岁以下儿童出现了与其他临床特征明显相关的可识别综合征。这两种综合征,即高计数T细胞急性淋巴细胞白血病和高计数婴儿无免疫球蛋白轻链急性淋巴细胞白血病,是替代疗法的合理候选对象。确诊的脑膜疾病仍然是一个重大的治疗挑战。