向编辑:
We read with interest the article by Warth等等。1on mediastinal angiomyolipoma and tuberous sclerosis. Apart from mediastinal angiomyolipoma, lymphangioleiomyomatosis (LAM) can occur as part of the genetic condition tuberous sclerosis2。自Cornog和Eettline的第一个报告以来3.,LAM越来越多地研究了非典型平滑肌脉冲增殖的病理过程。然而,由于其罕见,难以完全了解林的自然历史。林倾向于缓慢进展,最终导致呼吸衰竭。尽管如此,林患者的临床进程可以变化,10年生存率在10-60%之间2。一些患者在演示后恢复了20岁2。LAM may remain silent until spontaneous pneumothorax occurs, which is the most frequently presenting symptom4.。这种情况下血尿病的适应症并不确定。在我们的经验中,通常推荐用于复发性或自发性双侧气胸的患者的外科尿素瘤,但是这组患者可以预期更高的复发率4.。此外,手术胸膜瘤后的胸膜异构化可能会使未来的肺移植不可能。可以术中观察多个漫射肿块(图1⇓),提醒临床医生对此条件。
经典地,计算断层扫描(CT)扫描显示在两个肺部分布的多个薄壁囊肿,尽管已经描述了单侧肺参与5.。CT扫描也有助于检测其他毫无戒心的病理学6.。Pathological examination is required for confirmation of LAM, which can be obtained by transbronchial, percutaneous or surgical biopsy. We advocate video-assisted thoracic surgery biopsy with excision of any bleb and culprit lesion, particularly in the case of associated pneumothorax. The lung is characterised by cystic changes associated with proliferation of atypical smooth muscle cells (LAM cells), which can involve bronchioles, vessels and airspaces explaining, in part, the occurrence of pneumothorax, chylothorax and haemoptysis in these patients. (fig. 2⇓) Positive HMB-45 immunostaining and the presence of oestrogen and progesterone receptors are also characteristics of LAM. Despite reports of various therapeutic regimens, none offer a consistently effective response. Corticosteroids and cytotoxic agents usually provide little benefit. Medical hormonal therapy (progesterone, tamoxifen, luteinising hormone-releasing hormone agonist) and surgical hormonal therapy (oophorectomy, ovarian radiofrequency ablation) have been used with variable responses7.。
For end-stage lymphangioleiomyomatosis, lung transplantation can be an effective treatment option, but recurrences in the transplanted lung may occur following lymphangioleiomyomatosis cell migration7.。
兴趣表
None declared.
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