Analysis Of 8 Children With Multi-system Langerhans Cell Histiocytosis

Langerhans cell histiocytosis(LCH)is a rare proliferative disease of pathological Langerhans cells in children.Until now,its pathogenesis is unknown.Because of the difference on the primary site and involvement of risk organ,the major clinical manifestation and prognosis are varied.Therefore,most paediatricians might be confused on the diagnosis and treatment of it.Objective: To explore the clinical features and the characteristics on diagnosis and prognosis of multi-system Langerhans Cell Histiocytosis(LCH)in children,with the purpose of improving the clinicians' understanding on it.Methods: Collecting the clinical data on 8 children with multi-system LCH treated in Department of Pediatric Oncology,the First Hospital of Jilin University from February 2013 to November 2015,and reviewing the updated materials on LCH,a retrospective clinical trial was made,focusing on the clinical features,treatment outcome and prognosis.Results: 8 children were enrolled in the clinical trial,2 females(2/8,25%)and 6 males(6/8,75%).The median age was 25 months.Among them,2 children were newborns(2/8,25%),5 children infants(5/8,62.5%).Major clinical manifestations included bone involvement(7/8,87.5%),lung involvement(6/8,75%),rash(5/8,62.5%),liver dysfunction(3/8,37.5%),ear involvement(3/8,37.5%),lymphadenopathy(3/8,37.5%),diabetes insipidus(1/8,12.5%).The initial treatment response rate was 100%.Treatment-related toxicities,especially in infant,were mainly ? °-? ° myelosupression and ? °-? ° liver dysfunction,mild in elder children.Abnormal manifestations on gastric tract were mild.4 cases(4/8,50%)presented progressive diseases during therapy,2 cases(2/7,28.6%)presented reactive disease during follow-up.The event-free survival rate was 71.4%.Overall survival rate was 87.5%.The median follow-up time was 13.3 months.Children with progressive disease or reactive disease also presented good response to chemotherapy containing etoposide.Conclusion: In this trial,younger children with multi-system LCH presented with higher incidence and better prognosis,but often accompanying with progressive disease or reactive disease,which could be controlled by the intensive chemotherapy.