OBSTRUCTIVE LUNG-DISEASE AFTER ALLOGENEIC MARROW TRANSPLANTATION - CLINICAL PRESENTATION AND COURSE
OBSTRUCTIVE LUNG-DISEASE AFTER ALLOGENEIC MARROW TRANSPLANTATION - CLINICAL PRESENTATION AND COURSE
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DOI：
10.7326/0003-4819-111-5-368
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发表时间：
1989-09-01
影响因子：
39.2
通讯作者：
SULLIVAN, KM
中科院分区：
文献类型：
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作者：
CLARK, JG;CRAWFORD, SW;SULLIVAN, KM
为了描述骨髓移植后阻塞性肺疾病的临床表现和进展，我们对35例在1980年1月至1987年1月间接受同种异体骨髓移植的患者进行了连续研究，这些患者年龄在16岁或以上，移植前肺功能检查正常，移植后50天或更长时间出现气流阻塞，定义为FEV1/FVC低于70%，FEV1低于80%。病例选自同期接受同种异体骨髓移植的1029例成人（16岁以上）患者。气流阻塞患者表现为咳嗽、呼吸困难或喘息，或两者兼有。80%胸片正常。35例患者中有33例在移植后1.5年内诊断出气流阻塞。24例患者出现临床、广泛、慢性移植物抗宿主病。只有4例患者对慢性移植物抗宿主病的初步治疗有完全反应。血清IgG和IgA水平分别下降15例和25例。21例患者FEV1迅速下降（两次检测之间FEV1下降30%），但14例患者被确定为缓慢进展或可逆性疾病。移植后3年死亡率为65%，显著高于对照组412例16岁及以上、移植后存活超过80天且肺功能正常的慢性移植物抗宿主病患者的3年死亡率44% （P = 0.016）。我们的结论是，骨髓移植后的阻塞性肺疾病在发病时间和进展速度方面可能是可变的。阻塞性肺疾病通常与血清免疫球蛋白缺乏和临床广泛的慢性移植物抗宿主病相关，这种疾病不易对治疗产生反应。死亡率很高，但确定了长期幸存者。
To describe the clinical presentation and progression of obstructive lung disease after marrow transplantation, we examined a sequential sample of 35 patients who had allogeneic marrow transplantation between January 1980 and January 1987, were 16 years or older, had normal pulmonary function tests before transplantation, and developed airflow obstruction defined as FEV1/FVC less than 70% and FEV1 less than 80% predicted 50 days or more after transplantation. Cases were selected from 1029 adult (older than 16 years) patients who underwent allogeneic marrow transplantation during the same period. Patients ith airflow obstruction presented with symptoms of cough, dyspnea, or wheezing, or a combination. In 80% the chest radiograph was normal. Airflow obstruction was diagnosed within 1.5 years after transplantation in 33 of 35 patients. Clinical, extensive, chronic graft-versus-host disease was present in 24 patients. Only 4 patients had a complete response to primary therapy of chronic graft-versus host disease. Serum IgG and IgA levels were decreased in 15 and 25 patients, respectively. The FEV1 declined rapidly (decrease in FEV1 > 30% between tests) in 21 patients, but 14 patients with slowly progressive or reversible disease were identified. Mortality was 65% at 3 years after transplant, a significant higher value (P = 0.016) than the 3-year mortality rate of 44% in a comparison group of 412 concurrent patients with chronic graft-versus-host disease who were 16 years or older, survived more than 80 days after transplantation, and had normal pulmonary function. We concluded that obstructive lung disease after marrow transplantation may be variable with respect to time of onset and rate of progression. Obstructive lung disease was frequently associated with serum immunoglobulin deficiency and clinical, extensive, chronic graft-versus-host disease that was not readily responsive to treatment. Mortality was high but long-term survivors were identified.