Ladisch S, Gadner H
Center for Cancer and Transplantation Biology, Children's Research Institute, Washington, D.C. 20010-2970.
Br J Cancer Suppl. 1994 Sep;23:S41-6.
Optimal treatment of Langerhans cell histiocytosis remains problematic. The absence of controlled studies and the lack of standard diagnostic and evaluation criteria have impeded therapeutic progress even though knowledge of basic aspects of LCH have advanced. Historical analysis of outcome suggests little improvement until very recently, but marked differences in outcome according to extent of disease. Consequently, major and now successful efforts have been made to stratify LCH patients into different 'risk groups'. Recent findings suggest that combination chemotherapy for multisystem disease is beneficial and that VP-16 is a useful new agent for treating LCH, despite controversies regarding its side effects. The first randomised international treatment study, LCH-1, being conducted by the Histiocyte Society, should resolve some of these controversies. Other experimental therapies may be considered for children with severe, unresponsive LCH.
朗格汉斯细胞组织细胞增多症的最佳治疗方案仍然存在问题。尽管对朗格汉斯细胞组织细胞增多症基本方面的认识有所进展，但缺乏对照研究以及标准的诊断和评估标准阻碍了治疗进展。对治疗结果的历史分析表明，直到最近才有了一些改善，但根据疾病程度不同，治疗结果存在显著差异。因此，人们做出了重大且目前已取得成功的努力，将朗格汉斯细胞组织细胞增多症患者分为不同的“风险组”。最近的研究结果表明，多系统疾病采用联合化疗是有益的，并且依托泊苷是治疗朗格汉斯细胞组织细胞增多症的一种有用新药，尽管其副作用存在争议。组织细胞协会正在进行的第一项随机国际治疗研究LCH-1，应该能解决其中一些争议。对于患有严重、无反应性朗格汉斯细胞组织细胞增多症的儿童，可考虑其他实验性疗法。
