Clinical Analysis And Follow-up Study Of84Cases Langerhans Cell Histiocytosis In Children

BackgroundLangerhans Cell Histiocytosis is a rare and varies disease,characterized by accumulation of dendritic cells which similar to epidermalLangerhans cells. The pathogenesis of LCH is still unclear. Currently, italso has a debate on it’s an active disease or a neoplastic disease. LCHoften affects children, and it can affects any tissue or organ, including theskeleton, skin, liver, spleen, lungs, the hematopoietic system and so on. Theclinical manifestation and prognosis can range from a self-limiting rash ormass to a fatal multiple organ failure.ObjectiveTo understand the clinical characteristics and prognosis of LCH inchildren.Subjects and methodsSelected the definite LCH patients of children’s hospital in Chongqingmedical university during2006.1.1to2013.12.31, review the medicalrecords and follow-up by telephone. Used SPSS20.0statistical software to complete the data processing.Result1.In the study,male/female ratio is1.33:1，the median age of diseaseonset is2years old and6days(range,0-15yr), the median time from diseaseonset to dignosis is1month(range,0-6y). In the patients of LCH, thepercentage of SS-LCH and MS-LCH respectively are54%and46%。56%patients with MS-LCH onset in less than1year old, and the SS-LCHpatients have symptoms after3years old account for67%.2. The most frequent symptoms in complaint are masses orswell(36cases), rash(18cases), fever(17cases), abnormal activity(16cases)and unexplained pain(12cases). In SS-LCH patients, the most commonsymptom is masses or swell; and rash is the most common symptom inMS-LCH patients.3. The most common involoved organ is skeleton (65cases,77%),followed by liver (29cases,35%) and skin (25cases,30%). The patientsonset less than1year old are common involoved with liver (72%) andskin (68%); however, skeleton(83%) and liver (38%) are commoninvoloved in the patients between1yr to3yr. The patients onset more than3years old are common involoved with skeleton (94%).4. The main treatment are surgery and chemotherapy, we make theappropriate plan according to the patient’s general condition, economiccondition, and so on. 5. In our study, the1yr overall surivival rate of all patients is93.8%,and the1yr overall surivival of SS-LCH and MS-LCH respectively are100%and86%(p=0.003).ConclusionIn children, the involved organ or system is associated with the onsetage of disease. Generally, the younger the easiler involved with skin andliver, the easiler developing to MS-LCH. In constrast, SS-LCH is morecommon affected older children. To the cutaneous LCH, the patient with awide range eruption is more likely to be MS-LCH. The age of disease onsetyounger than1yr and involved with organ or system are often related topoor prognosis. All LCH patients need to complete systemic exam andfollow-up lifetime.