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