Boehler A, Speich R, Russi E W, Weder W
Department of Internal Medicine, University Hospital of Zurich, Switzerland.
N Engl J Med. 1996 Oct 24;335(17):1275-80. doi: 10.1056/NEJM199610243351704.
Lymphangioleiomyomatosis is a rare disease of unknown origin that usually leads to progressive deterioration of lung function and eventual death from respiratory failure. It occurs in women of reproductive age and people with tuberous sclerosis. Lung transplantation is a recent therapeutic approach.
We conducted a retrospective study by questionnaire of 34 patients, treated at 16 transplantation centers, who underwent lung transplantation for end-stage lymphangioleiomyomatosis between 1983 and 1995.
Of the 34 patients, 27 received single-lung transplants; 6, bilateral transplants; and 1, a heart-lung transplant. As of August 31, 1995, the actuarial survival calculated by the Kaplan-Meier method was 69 percent after one year and 58 percent after two years. Eighteen patients were alive 33 +/- 20 months (range, 3 to 74) after transplantation. Forced expiratory volume in one second increased from 24 +/- 12 percent of the predicted value before transplantation to 48 +/- 16 percent six months after transplantation. Five early deaths (within one month) were due to hemorrhage (in one patient), acute lung injury (in three), and dehiscence of the bronchial anastomosis (in one). Eleven late deaths (after one month) were due to infections (in eight patients), bronchiolitis obliterans (in two), and metastatic nephroblastoma (in one). Disease-associated problems were extensive pleural adhesions in 18 patients, leading to moderate-to-severe intraoperative hemorrhage in 4; pneumothorax in the native lung after single-lung transplantation in 6 patients; postoperative chylothorax in 3; and recurrent lymphangioleiomyomatosis in the allograft in 1 patient, who died of disseminated aspergillosis.
Although disease-related complications are frequent, lung transplantation can be a valuable therapy for patients with end-stage lymphangioleiomyomatosis.
淋巴管平滑肌瘤病是一种病因不明的罕见疾病，通常会导致肺功能进行性恶化，最终死于呼吸衰竭。该病发生于育龄期女性和结节性硬化症患者。肺移植是一种近期采用的治疗方法。
我们通过问卷调查对1983年至1995年间在16个移植中心接受终末期淋巴管平滑肌瘤病肺移植治疗的34例患者进行了一项回顾性研究。
34例患者中，27例接受了单肺移植；6例接受了双侧肺移植；1例接受了心肺移植。截至1995年8月31日，采用Kaplan-Meier法计算的精算生存率为1年后69%，2年后58%。18例患者在移植后33±20个月（范围3至74个月）存活。一秒用力呼气量从移植前预测值的24±12%增加到移植后6个月的48±16%。5例早期死亡（1个月内）原因分别为出血（1例患者）、急性肺损伤（3例）和支气管吻合口裂开（1例）。11例晚期死亡（1个月后）原因分别为感染（8例患者）、闭塞性细支气管炎（2例）和转移性肾母细胞瘤（1例）。与疾病相关的问题包括18例患者有广泛的胸膜粘连，其中4例导致术中出现中至重度出血；6例患者单肺移植后出现患侧气胸；3例患者术后出现乳糜胸；1例患者移植肺出现复发性淋巴管平滑肌瘤病，死于播散性曲霉病。
尽管与疾病相关的并发症很常见，但肺移植对于终末期淋巴管平滑肌瘤病患者可能是一种有价值的治疗方法。
