Report Of A Boy With Diabetes Insipidus And Langerhans Cell Histiocytosis,and Literature Review

Objective Langerhans histiocytosis(LCH)is a rare disease characterized by the proliferation of specific dendritic cells in the monocyte-phagocytic system.The etiology and pathogenesis of the disease have not yet been elucidated.the disease can involve multiple systems,and its clinical manifestations are diverse and lack of specificity.It is reported that the incidence of LCH is around 1/100,000,but is relatively low in adult,about 1/100 million.Therapy with chemotherapy is common practice,but precision therapy against BRAV 600 mutation is state of art.Several cases report with nonspecific but specially on over activation of macrophage,indomethacin,showed economic,adequate efficacy and less adverse side effects.We reported one boy who suffered from diabetes insipidus,but with uncommon manifestation of bones,eyes,teeth involvement.The diagnosis of LCH was confirmed after pathologic study of biopsy of skull tissue.Websites were searched for case reports in mainland,China.The clinical characteristics and treatment of adult LCH were systematically analyzed.Methods The clinical data of an adult LCH patient diagnosed pathologically in the author's department were analyzed retrospectively.The related cases of Langerhans cell histiocytosis in China from 2010 to 2018 were searched,such as China knowledge Network,Wan fang,Weipu Database,etc,and the related cases of Langerhans cell histiocytosis in China from 2010 to 2018 were searched.50 cases with complete recorded data and 1 case mentioned above were selected.a total of 51 cases were analyzed retrospectively.The ages,sex,classification,clinical manifestations and treatment regimens were analyzed retrospectively.The population distribution,clinical manifestations,diagnostic criteria,treatment and prognosis of the disease were discussed by reviewing other related literatures.Results A case of adult disease was reported.the first symptom was polydipsia and polyuria.the lesion involved pituitary gland,skin mucosa,bone and lymph node.it was diagnosed by skull pathological biopsy and immunohistochemistry.After treatment with desmopressin acetate and indomethacin,the condition was improved,and the follow-up condition was stable after two months of therapy.Of the 51 patients,39 were male and 12 were female.the average age of onset was 40.8 years,with a median age of 39 years.There were 15 cases of Single system Langerhans cell histiocytosis(SS-LCH)and36 cases of multisystem Langerhans cell histiocytosis(MS-LCH).9 cases had the first symptoms of bone pain,6 cases began with polydipsia,5 cases with skin mass,5 cases with cough and expectoration,5 cases with rash with pruritus,4 cases with lymphadenopathy,4 cases with jaundice,2 cases with fever,2 cases with upper abdominal pain,and 2 cases with gingival atrophy.There were only 1 case of vulvar ulcer,1 case of dizziness,1 case of headache,1 case of pneumothorax,1 case of chest pain,1 case of emaciation,and 1 case of gastric discomfort.The most commonly involved organs were bone(23 cases,45%),skin and mucosa in 20 cases(39.2%),lung in 19 cases(37.2%),lymph node involvement was found in 19 cases(37.2%),pituitary in 16 cases(31.4%),liver and spleen in 13 cases(25.5%),bone marrow in 11 cases(21.6%),thyroid in 3 cases(5.9%),kidney in 1 case(2.0%),and central nervous system in 1 case(2%).Most of the patients with SS-LCH were treated with symptomatic therapy alone,hormone alone or chemotherapy alone,and 66.7%of them had a good response.At present,the most common practice of therapy was systemic chemotherapy for MSLCH,and 61.1%achieved good response.The remission rate of SS-LCH was higher than that of MS-LCH,and the curative effect and prognosis were better.Conclusions LCH is a rare disease caused by the infiltration of monoclonal pathological Langerhans cells in different tissues and organs.The clinical manifestations of this disease are various.the symptoms involve many clinical departments,which are easy to be missed,the diagnosis is mainly depended on pathomorphological examination and immunohistochemical examination.Cooperation and communication among various medical disciplines should be strengthened to improve the diagnosis of LCH.The possibility of LCH should be considered when there are clinical manifestations such as bone destruction,rash,lung imaging changes,lymphadenopathy,especially in patient companied with diabetes insipidus.Because the involved organs and systems are very different,the clinical manifestations are different,and the treatment schemes are different,it is very important to carry out individualized treatment of adult LCH.At present,the traditional chemotherapy drugs are mainly used in the treatment,and the research and application of the new generation of drugs is the mainstream direction of the next step of clinical research.The study of the pathogenesis of LCH and large-scale clinical trials are helpful to improve the understanding of the disease,reduce misdiagnosis,missed diagnosis and improve the survival rate.