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目的:分析异基因造血干细胞移植后阻塞性肺病的临床特点和发病因素。方法:厦门大学附属中山医院血液科于2002-12/2005-04对27例血液病患者进行了异基因造血干细胞移植。移植前均无肺部疾患史,胸部X射线检查肺功能均正常。随诊截止时间为2006-04-30。预处理均采用以马利兰+环磷酰胺为基础的方案,预防移植物抗宿主病采用骁悉、环孢素及短程甲氨喋呤方案,移植前给予更昔洛韦预防巨细胞病毒感染。阻塞性肺疾病诊断标准:有呼吸道症状(干咳、呼吸困难)但无严重感染证据,肺功能指标:1s用力呼气量小于预计值的80%及1s用力呼气量/用力肺活量小于80%,胸部X线片显示可正常或过度通气,高分辨CT可表现为弥漫性肺泡实变,支气管、细支气管扩张。分析患有阻塞性肺病患者的造血功能重建情况、移植物抗宿主病发生情况、阻塞性肺病的发生情况、治疗及预后情况。结果:27例患者均进入结果分析。①根据临床诊断标准,7例患者患有阻塞性肺病,发生率为26%,中位发病时间为移植后300d(110~390d),均伴有慢性移植物抗宿主病。②主要表现为干嗽(57%),呼吸困难、双肺呼吸音弱(100%),胸片未见异常,胸部高分辨CT分别显示正常、下肺少量较淡的模糊小片状影及支气管扩张;肺功能均显示重度混合性通气功能障碍。③抗生素治疗无效,免疫抑制剂及激素治疗有效,但于减量或停药后症状可加重,3例死于呼吸衰竭,4例症状好转,但肺功能无改善。结论:阻塞性肺病是移植后严重并发症之一,部分为进行性发展,死亡率较高,定期的肺功能检查有助于早期诊断;其原因可能主要为非感染性因素所致。
Objective: To analyze the clinical features and pathogenesis of obstructive pulmonary disease after allogeneic hematopoietic stem cell transplantation. Methods: Allogeneic hematopoietic stem cell transplantation was performed in 27 hematological patients from December 2002 to April 2005 in Department of Hematology, Zhongshan Hospital Affiliated to Xiamen University. No history of pulmonary disease before transplantation, chest X-ray examination of lung function were normal. The deadline for follow-up is 2006-04-30. Pretreatment are based on the use of marylan + cyclophosphamide-based programs to prevent graft-versus-host disease using cell-mediated, cyclosporine and short-course methotrexate program, given before ganciclovir cytomegalovirus infection. Obstructive pulmonary disease diagnostic criteria: respiratory symptoms (dry cough, dyspnea) but no evidence of serious infection, pulmonary function indicators: 1s forced expiratory volume less than 80% of the expected value and 1s forced expiratory volume / forced vital capacity less than 80% Chest X-ray showed normal or hyperventilation, high resolution CT can be manifested as diffuse alveolar consolidation, bronchial, bronchiectasis. Analysis of patients with obstructive pulmonary disease reconstruction of hematopoietic function, graft-versus-host disease, obstructive pulmonary disease, treatment and prognosis. Results: All 27 patients entered the result analysis. According to the clinical diagnostic criteria, 7 patients with obstructive pulmonary disease, the incidence was 26%, the median onset of 300 days after transplantation (110 ~ 390d), are associated with chronic graft-versus-host disease. The main manifestations of dry cough (57%), dyspnea, lung breath sounds weak (100%), no abnormal chest X-ray, chest high resolution CT showed normal, the lower lung a small amount of light fuzzy patchy and Bronchiectasis; pulmonary function showed severe mixed ventilatory dysfunction. ③ antibiotics ineffective, immunosuppressive agents and hormone therapy effective, but after reduction or withdrawal symptoms may aggravate, 3 patients died of respiratory failure, 4 patients improved, but no improvement in lung function. Conclusions: Obstructive pulmonary disease (COPD) is one of the serious complications after transplantation. Some of them are progressive development with high mortality rate. Regular pulmonary function tests are helpful for early diagnosis. The reason may be mainly non-infectious factors.