成人多系统朗格汉斯细胞组织细胞增生症1例并文献复习
Multisystemic Langerhans cell histiocytosis in adults:a case report and literature review
刘焕君 1郭树霞 1石瑞平 1张文邦 1张晓娟1
作者信息
- 1. 河南中医药大学附属第五临床学院(郑州人民医院)血液科,河南 郑州 450003
- 折叠
摘要
目的 对 1 例全身多系统受累的朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)患者的诊疗过程进行报道以及对相关文献进行复习,提高临床工作者对此病的认识,为疾病的治疗提供方案.方法 回顾性收集并分析 1 例LCH患者的临床资料,并对相关文献进行检索复习.结果 从颈椎棘突、淋巴结、结肠等部位组织中检测到CD1a(+)、S100(+)、Langerin(+),患者确诊为LCH.经过 3 周期"克拉屈滨+阿糖胞苷"、3 周期"克拉屈滨"强化治疗、12 周期"长春地辛+强的松"巩固治疗,以及后续"来那度胺"的维持治疗 2 年.院外随访 2年,患者病变明显消退.结论 BRAF V600E突变阴性的患者使用"克拉屈滨+阿糖胞苷,长春地辛+强的松,以及后续来那度胺的维持治疗"是治疗难治性LCH患者的有效化疗方案.
Abstract
Objective To report the diagnosis and treatment of a case of multisystemic Langerhans cell histiocytosis(LCH)and review relevant literature,so as to improve the knowledge of clinicians about this disease and provide treat-ment plans.Methods The clinical data of a LCH case were retrospectively analyzed,and relevant literature was reviewed.Results The diagnosis of LCH was confirmed through the detection of CD1a(+),S100(+),and Langerin(+)in cervical spine spinoprocess,lymph nodes and colon tissues.After 3 cycles of cladribine+cytarabine,followed by 3 cycles of intensive cladribine treatment,12 cycles of vindesine+prednisone consolidation treatment,and 2-year mainte-nance treatment with lenalidomide,the lesions significantly subsided over a 2-year follow-up.Conclusion The regimen of cladribine+cytarabine,vindesine+prednisone,and subsequent lenalidomide maintenance therapy is an effective chemotherapy for refractory LCH patients with BRAF V600E negative mutation.
关键词
朗格汉斯细胞组织细胞增生症/多系统/肝脏/克拉屈滨/长春地辛Key words
Langerhans cell histiocytosis/Multiple systems/Liver/Cladribine/Vindesine引用本文复制引用
基金项目
河南省重点研发与推广专项(212102310791)
出版年
2024
NETL
NSTL
万方数据