Clinical Characteristics And Therapeutic Analysis Of Langerhans Cell Histiocytosis Of120Cases

OBJECTIVE：Analyse the clinical characteristics and treatment oflangerhans cell histiocytosis（LCH）for the clinical doctor understandingthe disease well in diagnosis and treatment.METHODS：A retrospective study was performed on the patientsdiagnosed LCH at the Departments of the Affiliated First Hospital ofGuangxi Medical University and the Affiliated Tumor Hospital ofGuangxi Medical University between January1,2006to December31,2013. The analysis included the gender, age, classification, therapeuticoutcome.RESULTS: Of the120patients,77were male and43were female(ratio of1.79:1).Median age was8.3years. There were36patients insingle-system langerhans cell histiocytosis (SS-LCH) and39inmultisystem langerhans cell histiocytosis (MS-LCH) of the youth, while 28in SS-LCH and17in MS-LCH of the adults. There were21patientswith risk organ dysfunction in MS-LCH and83.3%of them were youngerthan2years with risk organ dysfunction. The chief complaint atpresentation were lesion mass(50), pain(45), rash(11), limping gait(10),fever(7), discharging ear(4), blurred vision(2), diarrhea(1), jaundice(1),anhelation(1), diuresis(1). In SS-LCH group, there were84.3%ofpatients in surgery only and93.5%of them achieved better response. InMS-LCH group, there were64.2%of patients in systemic chemotherapyand77.1%of them achieved better response.10patients relapsed (4patients in SS-LCH, while6patients in MS-LCH). In the survivalanalysis, it showed that the classification, grading (It graded in age,number of organs, with/without risk organ dysfunction) were associatedwith prognosis (P <0.05).CONCLUSIONS:1.LCH occurred frequently in teenagers.SS-LCH was more common than MS-LCH in the youth, while oppositein adults. Risk organs dysfunction were easily involved in the patientswho were younger than2years in MS-LCH.2. There were kinds of clinical manifestations that were associated withdifferent disciplines. It was good for improving the diagnosis andtreatment level of the doctors in LCH by strengthening thecommunication and cooperation between different disciplines.3. The drugs used in LCH were the traditional drugs at present. The research of new drugs in LCH will be the direction of the future research.4. They were associated with the prognosis in the classification, age, thenumber of organs dysfunction and with/without risk organ dysfunction...