Boehler A
Division of Pulmonary Medicine and Lung Transplant Program, University Hospital, Zurich, Switzerland.
Semin Respir Crit Care Med. 2001 Oct;22(5):509-16. doi: 10.1055/s-2001-18423.
Lung transplantation for respiratory failure due to lymphangioleiomyomatosis, histiocytosis X, and sarcoidosis is a therapeutic option in selected patients. These indications account for less than 6% of all lung transplant procedures, and experience is relatively scarce. Pulmonary function testing at evaluation usually shows an obstructive pattern in lymphangioleiomyomatosis, whereas histiocytosis presents with a mixed pattern, and sarcoidosis with a restrictive pattern. Lung diffusion capacity is similarly reduced, however, only histiocytosis and sarcoidosis are often associated with pulmonary hypertension. In all three diseases extrapulmonary manifestations, although of different kinds, are common and must be investigated before transplantation to avoid posttransplant diagnostic pitfalls. In all these diseases, single and bilateral transplantation show comparable results. Typical complications in lymphangioleiomyomatosis include intraoperative severe intrathoracic bleeding, posttransplant chylothorax, pneumothorax, and bleeding of angiomyolipomas. In histiocytosis, extrapulmonary manifestations in bones and pituitary gland may progress. These diseases typically recur in the allograft, leading to clinical symptoms in histiocytosis whereas recurrence in lymphangioleiomyomatosis and sarcoidosis is most often asymptomatic. Survival is similar to patients transplanted for other diseases.
对于因淋巴管平滑肌瘤病、组织细胞增多症X和结节病导致呼吸衰竭的患者，肺移植是一种可供选择的治疗方法。这些适应症在所有肺移植手术中占比不到6%，相关经验相对较少。评估时的肺功能测试通常显示，淋巴管平滑肌瘤病呈阻塞性模式，而组织细胞增多症呈混合性模式，结节病呈限制性模式。肺弥散能力同样降低，不过，只有组织细胞增多症和结节病常伴有肺动脉高压。在这三种疾病中，肺外表现虽然各不相同，但很常见，移植前必须进行检查，以避免移植后出现诊断失误。在所有这些疾病中，单肺移植和双肺移植的效果相当。淋巴管平滑肌瘤病的典型并发症包括术中严重的胸腔内出血、移植后乳糜胸、气胸以及血管平滑肌脂肪瘤出血。在组织细胞增多症中，骨骼和垂体的肺外表现可能会进展。这些疾病通常会在移植肺中复发，导致组织细胞增多症出现临床症状，而淋巴管平滑肌瘤病和结节病的复发大多无症状。其生存率与因其他疾病接受移植的患者相似。
