Department of Medicine I, Center of Hematology an Stem Cell Transplantation, Hemostasis and Medical Oncology Internal Medicine I, Elisabethinen Hospital, Fadinger Str, 1 4010, Linz, Austria.
Orphanet J Rare Dis. 2013 May 14;8:72. doi: 10.1186/1750-1172-8-72.
Langerhans Cell Histiocytosis (LCH) is an orphan disease of clonal dendritic cells which may affect any organ of the body. Most of the knowledge about the diagnosis and therapy is based on pedriatic studies. Adult LCH patients are often evaluated by physicians who focus on only the most obviously affected organ without sufficient evaluation of other systems, resulting in patients being underdiagnosed and/or incompletely staged. Furthermore they may be treated with pediatric-based therapies which are less effective and sometimes more toxic for adults. The published literature on adult LCH cases lacks a comprehensive discussion on the differences between pediatric and adult patients and there are no recommendations for evaluation and comparative therapies. In order to fill this void, a number of experts in this field cooperated to develop the first recommendations for management of adult patients with LCH. Key questions were selected according to the clinical relevance focusing on diagnostic work up, therapy, and follow up. Based on the available literature up to December 2012, recommendations were established, drafts were commented by the entire group, and redrafted by the executive editor. The quality of evidence of the recommendations is predominantly attributed to the level of expert opinion. Final agreement was by consensus.
朗格汉斯细胞组织细胞增生症（LCH）是一种克隆性树突细胞的孤儿病，可能影响身体的任何器官。大多数关于诊断和治疗的知识都是基于儿科研究。成人 LCH 患者通常由仅关注最明显受影响器官的医生进行评估，而没有对其他系统进行充分评估，导致患者漏诊和/或分期不完整。此外，他们可能接受基于儿科的治疗，这些治疗对成人的疗效较差，有时毒性更大。关于成人 LCH 病例的已发表文献缺乏对儿科和成人患者之间差异的综合讨论，也没有关于评估和比较治疗的建议。为了填补这一空白，该领域的一些专家合作制定了第一份成人 LCH 患者管理建议。根据临床相关性选择关键问题，重点关注诊断工作、治疗和随访。根据截至 2012 年 12 月的现有文献，制定了建议，草案由全体小组成员进行了评论，并由执行编辑重新起草。建议的证据质量主要归因于专家意见的水平。最终通过协商达成一致。
