Journal of Shandong University (Health Sciences) ›› 2024, Vol. 62 ›› Issue (2): 83-89.doi: 10.6040/j.issn.1671-7554.0.2023.0961

• Clinical Medicine • Previous Articles    

Multisystemic Langerhans cell histiocytosis in adults: a case report and literature review

LIU Huanjun, GUO Shuxia, SHI Ruiping, ZHANG Wenbang, ZHANG Xiaojuan   

  1. Department of Hematology, The Fifth Clinical College Affiliated to Henan University of Chinese Medicine (Zhengzhou Peoples Hospital), Zhengzhou 450003, Henan, China
  • Published:2024-03-29

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

CLC Number: 

  • R551.3
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