Clinical Analysis Of Combined Chemotherapy With Cytarabine For Refractory Langerhans Cell Histiocytosis

Objective: To evaluate the efficacy of combined chemotherapy with cytarabine(Ara-C)for children with refractory Langerhans cell histiocytosis(LCH).Methods: The clinical data of patients with LCH in Children’s Hospital of Chongqing Medical University from January 1st,2013 to December 31 st,2018 were collected,and followed up to February 29 th,2020.The refractory LCH patients treated with combined Ara-C chemotherapy were analyzed retrospectively,the treatment response was evaluated and the curative effect was analyzed.Results: A total of 11 patients with refractory LCH were included,with a male-to-female ratio of 6 to 5,their median diagnostic age was 22 months,median follow-up duration was 32 months.And all of the patients were MS-LCH,10 patients had central nervous system(CNS)involvement,1 patient had risk organ involvement(spleen).After first-line treatment,9 patients had disease progression(8 patients had skeletal progression),and 2 patients had stable disease.All patients were treated with combined Ara-C chemotherapy.The overall response rate was 91%(1 patient was cured,9 patients were improved,and 1 patient was in a stable state),the overall survival rate was 100%.For patients with CNS involvement(n=9),5/9 patients had a complete regression of the CNS mass.And 2 patients had sequelae,both of which were diabetes insipidus.Conclusions: The effect of first-line treatment in refractory patients is not ideal,and it is easy to relapse,in which bone is the common site of recurrence of LCH.Ara-C combined with chemotherapy for refractory LCH had a high response rate and a significant effect;it also had a better effect for patients with CNS involvement.