| Objective:To improve the understanding of bronchiolitis obliterans syndrome?BOS?after hematopoietic stem cell transplantation?HSCT?.Methods:A review of 997 patients who underwent HSCT in the First Affiliated Hospital of Guangxi Medical University from January 2003 to April2019 was conducted,the clinical data of two cases diagnosed with BOS after HSCT was analyzed.And relevant literatures were searched by databases such as cnki,wanfang,weipu and Pubmed to summarize the clinical characteristics of BOS after HSCT.Results:?1?Cases data of our hospital:Two cases both are middle-aged men combined with chronic graft-versus-host disease?cGVHD?.The time from HSCT to BOS was 4.2 months and 23.2 months respectively,developping to BOS after stopping anti-rejection drugs for more than 1 month.The clinical manifestations were shortness of breath after activity,accompaning by cough and expectoration.Pulmonary function showed obstructive ventilation dysfunction including one severe case and one moderate case.Lung CT of case1 showed mosaic sign and air trapping,lung CT of case 2 showed air trapping.The first diagnosis of two cases was pneumonia,cough and expectoration were improved after anti-infection treatment,but there was no significant improvement in shortness of breath.Treatment of immunosuppressor,macrolide antibiotics and bronchodilators were be used after the diagnosis of BOS,the symptoms of shortness of breath was improved after the above treatment,patients were followed up for stable condition.?2?Literature review:A total of sixty BOS after HSCT literatures,including ten Chinese literatures and fifty English literatures,were retrieved and screened out from January 1975 to April2019,991 cases were counted according to the diagnostic criteria.Among them,538 cases were male,406 cases were female,47 cases did not provide a gender,average age was 41.1±16.7 years old?6-64 years old?.The average time from HSCT to BOS was 15.8±6.2 months?1.6-168 months?,59%patients developped to BOS at 12-24 months after HSCT,a very small number?0.2%?patients appeared within 3 months after HSCT.720 cases?72.7%?were combined with cGVHD.84.4%patients presented with varying degrees of shortness of breath symptoms,the second was cough,expectoration and so on.57.8%patients'pulmonary function showed moderate obstructive ventilation dysfunction,78.8%patients'pulmonary function showed mild diffuse dysfunction.The main manifestation of lung imaging were air retention?64.4%?,bronchial wall thickening?33.3%?and bronchiectasis?31.2%?.Pulmonary histopathology showed bronchiolar fibrosis,bronchiolar stenosis and occlusion.Treatment methodsincludedimmunosuppressiveagents,macrolideantibiotics,bronchodilators,and lung transplantation in patients with end-stage lung function.After treatment,the overall average value FEV1pred%was 10.9%higher than that at diagnosis of BOS?P<0.001?,as well as FEV1/FVC%and DLCO%pred?P<0.001?.641 cases provided follow-up,improved in 37cases?5.8%?,stabilized in 381 cases?59.4%?,pejorative in 6 cases?1.0%?,dead in 217 cases?33.9%?.Among them,42 cases died of respiratory failure,17 cases died of recurrence of primary blood system diseases,and 14 cases died of infection,the remaining causes were not specified.Conclusions:?1?The occurrence of BOS after HSCT may be related to the withdrawal of anti-rejection drugs.?2?Shortness of breath after activities,obstructive ventilation dysfunction,air retention and combined with cGVHD are common clinical features of the vast majority of BOS after HSCT patients.?3?Lung function examination before HSCT and every 3 months after HSCT,or at the time of cGVHD and shortness of breath after activities after HSCT can find lesions as early as possible and reduce the misdiagnose and missed diagnosis.?4?The main treatment for this disease are immunosuppressor combined with macrolide antibiotics and bronchodilators,early therapeutic response is good,active treatment in late stage can maintain stable condition. |
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