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作者报导儿童慢性粒细胞白血病(CML)急变6例。5例Ph~1染色体(+);1例Ph~1(-),由于无皮疹、淋巴结肿大和出血倾向,故仍诊断为Ph~1(-)的成人型,而非幼年型CML。对急变是接1971年Canellos等所拟诊断标准判断。6例中有1例初诊时已呈慢粒急变,其他5例之慢性期为3至32月;取马利兰治疗,仅1例加用过6—巯基嘌呤。每例均在急变时检测骨髓有核细胞之末端脱氧核苷酸转移酶(TdT)活性,结果3例为(+),3例(-)。3例TdT(+)的原始细胞外形均似淋系;而3例(-)患者中,有2例原始细胞外形似粒系,另1例则呈粒—淋混合型。急变期均用长春新碱+强的松龙(VP)疗法,前者用1～2mg每周1次静注;后者口服60mg/日。有3例加用环磷酰胺与阿糖胞苷;1例用过红比霉素+阿糖胞苷;
The authors report 6 children with chronic myeloid leukemia (CML) sudden change. Five cases of Ph~1 chromosome (+) and one case of Ph~1(-) were diagnosed as Ph~(-) adult rather than juvenile CML because of no rash, lymphadenopathy, and bleeding tendency. The change was judged by Canellos et al.’s proposed diagnostic criteria in 1971. One of the 6 cases had a sudden change of the granulocytic gland at the time of initial diagnosis, and the other 5 cases had a chronic phase of 3 to 32 months. In the case of the marilean treatment, only 6 cases were added with 6-mercaptopurine. In each case, the terminal deoxynucleotidyl transferase (TdT) activity of bone marrow nucleated cells was detected at the time of rapid change. The results were 3 cases (+) and 3 cases (-). The appearance of the original TdT(+) cells in all three cases was similar to that of the lymphoid line. In the three cases (-), two of the original cells had an appearance like a granular system, and the other one showed a granular-lyophilic mixture. In the blast phase, vincristine plus prednisolone (VP) therapy was used. The former used 1 to 2 mg of intravenous injection once per week; the latter took 60 mg/day orally. Cyclophosphamide and cytarabine were added in 3 cases; red bromycin + cytarabine was used in 1 case;