朗格汉斯细胞囊细胞瘤成功地用thalidomide和皮质类固醇的组合治疗
Nadia Ayu Mulansari1,2, Abdul Muthalib1,2, Cynthia Kurniawan3
1Division of Haematology and Medical Oncology, Department of Internal Medicine, Faculty of Medicine, University of Indonesia, Jakarta, Indonesia.
European journal of case reports in internal medicine
|October 9, 2025
概括
朗格汉斯细胞囊细胞症 (LCH) 是一种罕见的疾病. 耐药性LCH病例可能会在标准治疗失败时从thalidomide和皮质类固醇联合治疗中受益.
科学领域:
- 在瘤学瘤学.
- 皮肤病学 皮肤病学
- 免疫学 免疫学 免疫学
背景情况:
- 朗格汉斯细胞胞症 (LCH) 是一种罕见的朗格汉斯细胞的克隆性疾病.
- 目前用于LCH的标准治疗方法在前性试验中缺乏经过证明的疗效,大约50%的患者没有反应.
- 对于成人LCH,特别是耐火病例,没有确立的黄金标准治疗方法.
研究的目的:
- 报告一个对标准治疗无反应的多器官LCH病例.
- 评估thalidomide和甲基prednisolone的潜力,作为耐火性LCH的替代治疗方法.
主要方法:
- 研究了一名51岁的女性患者,她患有多器官LCH,之前接受过维布拉斯和普雷迪尼索隆治疗,但没有完全反应.
- 患者接受了埃托波西德和甲基普雷迪尼索隆化疗与紫外线光疗.
- 随后的治疗包括thalidomide (每天100毫克) 和甲基prednisolone.
主要成果:
- 尽管初始治疗,但患者表现出持续的LCH,在PET/CT扫描上观察到进展.
- 皮肤病变,腹部节点和脊椎病变在标准化疗和光疗时没有显著改善.
- 用thalidomide和methylprednisolone治疗导致患者皮肤状况得到改善.
结论:
- 标准的LCH治疗在很大一部分成年患者中是无效的.
- 耐火性LCH缺乏推的标准治疗方案.
- thalidomide和皮质类固醇的组合为LCH患者提供了潜在的治疗选择,LCH患者对传统治疗没有反应.
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