Kalwinsky D K, Mirro J, Dahl G V
Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38101.
Pediatr Ann. 1988 Mar;17(3):172-6, 179-90. doi: 10.3928/0090-4481-19880301-07.
Childhood nonlymphocytic leukemia comprises a minority (25%) of pediatric leukemia cases, yet contributes a significant proportion of overall leukemia mortality. Improvements in supportive care (antibiotics, antifungals, nutrition, and blood products) along with aggressive induction therapies have significantly improved remission induction rates over the past two decades. Ideal treatment to completely eliminate residual disease following remission is not yet known. In most series, only one out of three patients are long-term survivors of this disease. Recent advances in allogeneic bone marrow transplantation and improved techniques for autologous engraftment leave promise for significant improvements in postinduction disease control. Biologic studies of surface immunophenotype have contributed to our understanding of the heterogeneity of this family of disorders and allowed identification and characterization of leukemias of mixed myeloid/lymphoid lineage. Karyotype studies have identified important subsets of ANLL with distinctive clinical and biologic properties, for which tailored therapies someday may be developed. In addition, studies of oncogenes provide insight into regulation of leukemic hematopoiesis with potential of identifying future methods to regulate proliferation of the leukemic clone.
儿童非淋巴细胞白血病占儿童白血病病例的少数(25%),但在白血病总死亡率中占相当大的比例。在过去二十年中,支持性治疗(抗生素、抗真菌药、营养和血液制品)的改善以及积极的诱导治疗显著提高了缓解诱导率。目前尚不清楚完全消除缓解后残留疾病的理想治疗方法。在大多数系列研究中,该疾病的长期幸存者仅占三分之一。异基因骨髓移植的最新进展以及自体移植技术的改进为诱导后疾病控制的显著改善带来了希望。表面免疫表型的生物学研究有助于我们理解这一疾病家族的异质性,并能够识别和表征混合髓系/淋巴细胞系白血病。核型研究已经确定了具有独特临床和生物学特性的急性非淋巴细胞白血病的重要亚组,未来可能会针对这些亚组开发量身定制的治疗方法。此外,癌基因研究有助于深入了解白血病造血的调控机制,有可能找到未来调控白血病克隆增殖的方法。