Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis
Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis
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DOI：
10.1182/blood-2012-09-455774
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发表时间：
2013-06-20
期刊：
影响因子：
20.3
通讯作者：
Ladisch, Stephan
中科院分区：
文献类型：
--
作者：
Gadner, Helmut;Minkov, Milen;Ladisch, Stephan
朗格汉斯细胞组织细胞增生症(LCH)-III测试了风险调整、强化、更长时间的多系统LCH(MS-LCH)治疗，对其最佳治疗一直难以捉摸。根据危险器官受累(高[RO+]或低[RO-]风险组)分层，>；400名患者被随机分组。RO+患者接受长春花碱+泼尼松(A组)或长春花碱+泼尼松+甲氨蝶呤(B组)治疗，疗程1~2个6周。应答触发了较温和的持续治疗，使用相同的组合，加上6-巯基嘌呤，共治疗12个月。两组6/12周有效率(平均71%)、5年生存率(84%)和复发率(27%)相似。值得注意的是，历史比较显示，在先前的试验LCH-I(62%)或LCH-II(69%)中，相同分层的RO+患者的存活率高于治疗6个月的患者(P
Langerhans cell histiocytosis (LCH)-III tested risk-adjusted, intensified, longer treatment of multisystem LCH (MS-LCH), for which optimal therapy has been elusive. Stratified by risk organ involvement (high [RO+] or low [RO-] risk groups), >400 patients were randomized. RO+ patients received 1 to 2 six-week courses of vinblastine+prednisone (Arm A) or vinblastine+prednisone+methotrexate (Arm B). Response triggered milder continuation therapy with the same combinations, plus 6-mercaptopurine, for 12 months total treatment. 6/12-week response rates (mean, 71%) and 5-year survival (84%) and reactivation rates (27%) were similar in both arms. Notably, historical comparisons revealed survival superior to that of identically stratified RO+ patients treated for 6 months in predecessor trials LCH-I (62%) or LCH-II (69%, P