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

doi:10.6040/j.issn.1671-7554.0.2023.0961

摘要/Abstract

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。

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

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 treatment 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 maintenance 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.

Key words: Langerhans cell histiocytosis, Multiple systems, Liver, Cladribine, Vindesine

中图分类号:

R551.3

刘焕君,郭树霞,石瑞平,张文邦,张晓娟. 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[J]. 山东大学学报 (医学版), 2024, 62(2): 83-89.

LIU Huanjun, GUO Shuxia, SHI Ruiping, ZHANG Wenbang, ZHANG Xiaojuan. Multisystemic Langerhans cell histiocytosis in adults: a case report and literature review[J]. Journal of Shandong University (Health Sciences), 2024, 62(2): 83-89.

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