The Clinical Analysis Of 73 Cases Adult Pulmonary Langerhans Cell Histiocytosis

AimPulmonary langerhans cell histiocytosis(PLCH)is a rare disease which has unclear pathogenesis,characterized by proliferation of langerhans cells that derived from bone marrow infiltrating the lungs and other organs,usually accompanied by inflammatory cell infiltration such as eosinophils,lymphocytes,and macrophages etc.Because the disease is relatively rare,currently,the understanding of the disease is insufficient,this paper aims to summarize the clinical characteristics and diagnosis and treatment of adult PLCH patients,to promote the understanding of the disease and help with the indiagnosis and treatment of the disease.Materials and methodsCollected 5 cases clinical data of adult PLCH patients(?18 years old)in our hospital,and searched adult PLCH cases on the database of Chinese journals CNKI?Wanfang,Pubmed and other journal databases published in the last five years,excluding the patients incidence age<18 years old,collected 68 cases with complete data and 5 cases of our hospital,a total of 73 cases,analyzed their clinical manifestations,laboratory and imaging examination,treatment and prognosis,promote understanding of the disease.ResultsAmong the 73 PLCH patients in this study,the youngest was 19 years old,the oldest was 76 years old,the median age was 38 years old,male 47 cases(64.4%),female 26 cases(35.6%).Smokers 58 cases(79.5%),include male 39 cases(53.4%),female 19 cases(26.0%),an average of 24 pack years,Smokers includ 6 cases(8.2%)previous smokers,lcases(1.4%)occupational exposure,2 cases(2.7%)cannabis user.15 cases(20.5%)non-smokers,1 case(1.4%)cigarette smog exposure,1 case(1.4%)of lampblack exposure and 1 case(1.4%)of occupational dust exposure.Standard classification recommended by the International Organization Association:49 cases(67.1%)of Single-system Pulmonary langerhans cell histiocytosis(SS-PLCH),24 cases(32.9%)of Multi-System Pulmonary langerhans cell histiocytosis(MS-PLCH)(6 cases involving 2 systems and above),12 cases of bone involvement(16.4%),6 cases of pituitary involvement(6.8%),6 cases of lymph node involvement(6.8%),3 cases of skin involvement(4.1%),2 cases of liver involvement(2.7%)and 1 case of thymus involvement(1.4%).The initial symptoms of 73 patients were mainly non-produced cough(47.9%)and dyspnea(45.2%),and some patients coughed with a small amount of white sputum.15 patients with pneumothorax as first manifestation was accounted for 20.5%of the total.5 cases(6.8%)showed pulmonary hypertension.Lung biopsy confirmed 53 cases(72.6%),bone biopsy 6 cases(8.2%),lymph node biopsy 5 cases(6.8%),skin confirmed 1 case(1.4%),liver 1 case(1.4%),bronchial lavage fluid CD1A cell>5%(6-8%)3 cases.Among the 73 patients,24 have not follow-up data,of the 49 patients with more detailed data,17 received smoking cessation treatment,13 improved after smoking cessation,10 cases were SS-PLCH patients,the lesions completely disappeared in 4 cases,4 patients progress after smoking cessation(include 2 MS-PLCH patients with pituitary involvement).Steroid drug therapy(0.5-1 mg/kg/days)was performed in 13 cases and improved in 3 cases(all were SS-PLCH),5.cases(including 2 cases of SS-PLCH),and 4 cases were still advanced(include 2 cases SS-PLCH).1 cases with pituitary involvement showed severe myasthenia discontinuation after oral administration of methylprednisolone(1 mg/kg/2 weeks).There were 7 cases of chemotherapy,2 cases were effective,that were MS-PLCH lung image improved,3 cases were stable(CHOP-E and CEOP chemotherapy regimen),1 case MS-PLCH lung lesion was stable,skin and lymph node lesion was improved(14 courses of cyclophosphamide+vincristine+dexamethasone),1 case MS-PLCH rectal anorectal lesion improved,lung lesion stability(5 cycles of vincristine+prednisone,14 cycles of cytarabine+vincristine and 8 cycles of gemcitabine+cis-platinum complexes),1 case liver lesion dissappeared and lung lesion stability(vincristine+prednisone).One of the patients who were misdiagnosed as rheumatoid arthritis,who treated after diagnosed with chemotherapy(glucocorticoid combined with vincristine,etoposide),died of pulmonary infection and sepsis.One SS-PLCH CT progress after smoking cessation,and chemotherapy(methotrexate,vincristine combined with methylprednisolone)was ineffective and died of pulmonary infection.All 8 patients received Cladribine treatment improved,including 6 cases SS-PLCH(including 1 case with advanced after smoking cessation,2 cases advanced after steroid treatment),2 effective cases showing leukopenia side effects.A SS-PLCH patient who progressd after treated by steroid was improved treated by the BRAF inhibitor Dabrafenib.Two cases of bone involvement MS-PLCH effective after Denosumab treatment,with pulmonary nodules reduced.One case without treatment improved,3 cases stable(all were SS-PLCH).3 cases of lung transplantation and 2 cases recurrence after transplantation,of which one SS-PLCH case did not quit smoking after transplantation,and the recurrent focus langerhans cells were identified from the receptor.One case of pulmonary lesion was misdiagnosed as pulmonary interstitial fibrosis,diagnosed after transplantation,determine skin and bone involvement,recurrence half a year after transplantation.Conclusion1.Adult PLCH are characterized by unproductive cough,dyspnea,the most age of onset is20-40 years old,its laboratory index is not specific,most of the chest images show pulmonary nodules and/or thin/thick wall cysts.2.PLCH patients who with spontaneous pneumothorax as initial manifestation were mostly young people.3.MS-PLCH patients smoke less than SS-PLCH patients,adults LCH patients who without pulmonary involvement with less smoking can cause lung involvement,it is recommended to quit smoking to avoid lung disease of them.4.The responsive of SS-PLCH patients and MS-PLCH patients to smoking cessation in pulmonary has no statistical difference.