| Objective:For providing direction for treatment,We explored the clinical characteristics,treatment and prognostic factors of Langerhans Cell Histiocytosis(LCH)in children,analyzed the efficacy of LCH-III approach and the prognosis of LCH treatment,evaluated and analyzed the overall survival rate and recurrence rate of LCH.Methods:We collected the medical records of 145 children admitted to the Children's hospital affiliated to Zhejiang University during January 2014 to May 2019 and diagnosed with Langerhans Cell Histiocytosis,retrospectively analyzed the records.According to the Histiocyte Society LCH-III approach,cases were divided into the single system involvement of Langerhans Cell Histiocytosis(SS-LCH),the multiple systems involved and with no risk organ involved Langerhans Cell Histiocytosis(MS-LCH RO-)and the multiple systems involved and with risk organ involved Langerhans Cell Histiocytosis(MS-LCH RO+),treated with vinblastine/prednisone,evaluated at 6 weeks,12 weeks of initial treatment and the final follow-up time,analysis of therapeutic effect and prognosis.Results:Among the group of 145 LCH cases,male were 77 cases,female were 68 cases(the ratio of male to female was 1.13:1.00),the median age at the time of diagnosis was 2.5 years old.After the evaluation,42 cases(29.0%)were single system involvement of Langerhans Cell Histiocytosis(SS-LCH),103 cases(71.0%)were multiple systems involved Langerhans Cell Histiocytosis(MS-LCH).The mass(40%)is the most common complaint symptom.In terms of treatment,single lesion(non-hazardous site)LCH patients accepted clinical observation,took Indomethacin or treated with vinblastine/prednisone according the Histiocyte Society LCH-III approach.Patients with single lesion(dangerous site or special site),multiple lesion of single skeletal system and multiple system involvement all accepted vinblastine/prednisone.Recurrence refractory cases accepted Ara-C+ Cladribine as salvage treatment.Among the 145 children,6 patients received observation and follow-up and oral treatment for anti-inflammatory,and 11 patients transferred to another hospital or abandoned therapy.128 patients received vinblastine/prednisone.110 cases with no active disease status after chemotherapy,10 cases had recurrence,and the recurrence rate was 6.9%(10/145),and 2 patients died(1 case refused chemotherapy;1 case Died of adverse reactions to chemotherapy).The condition was assessed after 6 weeks of treatment.Improvement rates were 70% at 6 weeks and 75% at 12 weeks.Survival analysis showed a 5-year survival rate of 100% for patients with single-system involvement(SS-LCH)and a low risk of recurrence,and a 5-year survival rate of 87.43% for patients with multi-system involvement(MS-LCH).Diabetes insipidus,is the most common sequela.The age at first diagnosis,maxillofacial bone involvement,the presence of organ dysfunction at first diagnosis have an impact on the prognosis.For relapsed and refractory children with LCH,the use of Cladribine + Ara-C regimen chemotherapy can achieve satisfactory efficacy.For children with positive BRAF gene,the efficacy of oral targeted drug remains to be verified.Conclusion:The peak age of LCH lesions in children is 1-3 years old,with more boys than girls.Bone,especially skull,is the most commonly affected part.Dangerous organ involvement and early treatment response are the key factors affecting prognosis.Early initiation of rescue plan,Cladribine combined with cytarabine arabinoside regimen chemotherapy has a good effect on refractory recurrent LCH in children,and targeted drug therapy has a good prospect?... |
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