Clinical analysis of 45 children with Langerhans cell histiocytosis

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 treatment: group 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 included: ①upgrading 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 relapse: liver (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. Key words: Langerhans cell histiocytosis; Chemotherapy; Child