Clinical analysis of 45 children with Langerhans cell histiocytosis / 中华实用儿科临床杂志

ABSTRACT

Objective To analyze the clinical data of children with Langerhans cell histiocytosis (LCH),to discuss the therapeutic effect,and to analyze the factors related to prognosis.Methods A total of 45 children diagnosed as LCH were divided into group A (18 cases with bone lesion only),group B(6 cases with soft tissue lesion),and group C (21 cases with viscera lesion) according to Shanghai Children's Hospital-LCH-2007 scheme [SCH-LCH-2007 (modified DAL-HX83/90) scheme].(1) Initial treatmentgroup A was treated with Prednisone (Pred) + Vincristine (VCR) for 28 weeks,and group B was treated with Pred + VCR + Etoposide (VP16) + Mercaptopurine (6MP) for 43 weeks,and group C was treated with Pred + VCR + VP16 + Methotrexate (MTX) +6MP for 52 weeks.(2) Re-treatment scheme after relapse included①upgrading treatment,group A to group B,group B to group C.②Individual treatment for group C included VP16 modification,and maintained Thymosin and/or Ciclosporin etc.Results The total survival rate was 93.3％ (42/45 cases) and recurrence rate was 26.7％ (12/45 cases).Children in group A and B were all effective,while 2 patients in group C died,and 1 case missed follow-up.Multi-factor analysis showed that the factors like age,sex,group,skeleton,soft tissue,erythra,lymph gland,lung,mouth,ears,hypophysis pituitary had no statistical significance,but liver,spleen and blood involvement had statistical significance in disease relapseliver (P=0.007 1),spleen (P=0.016 9),and blood (P=0.011 1).Conclusion LCH can affect several organs of children and relapse,and modified DAL-HX83/90 scheme is very effective.The liver,spleen and hematopoiesis system involvement is correlates with the relapse.