Ansótegui Barrera Emilio, Mancheño Franch Nuria, Peñalver Cuesta Juan Carlos, Vera-Sempere Francisco, Padilla Alarcón José
Servicio de Neumología, Hospital Universitario La Fe, Valencia, España.
Med Clin (Barc). 2013 Oct 19;141(8):349-52. doi: 10.1016/j.medcli.2013.05.036. Epub 2013 Aug 12.
Sporadic lymphangioleiomyomatosis (S-LAM) is a rare disease that affects only women. It is characterized by an abnormal proliferation of immature smooth muscle cells (LAM cells) that grow in an aberrant manner in the airway, parenchymal lung lymph and blood vessels, determining the onset of pulmonary cystic lesions. The disease has no treatment, progressing to respiratory failure, and lung transplantation (LT) may be a treatment option at this stage. Our goal was to study 7 patients undergoing LT for S-LAM.
We studied a series of clinical and demographic characteristics, diagnostic modality and post-transplant outcomes. We performed a descriptive analysis of the series. The Kaplan-Meier method was used to estimate survival.
The mean age of onset of symptoms was 35 years, the diagnosis of 37 years and that of LT 38 years. The most common symptom was dyspnea. Four patients had a history of pneumothorax and pleural effusion. The mean forced expiratory volume in one second was 32.7% and the diffusing capacity for carbon monoxide was 29%. All patients were subjected to LT and survival was 100, 85.7 and 57.1% at one, 3 and 5 years, respectively. Three died of bronchiolitis obliterans and 2 necropsies did not show evidence of disease recurrence.
LT is a therapeutic option in patients with S-LAM with an advanced respiratory functional impairment.
散发性淋巴管平滑肌瘤病（S-LAM）是一种仅影响女性的罕见疾病。其特征为未成熟平滑肌细胞（LAM细胞）异常增殖，这些细胞在气道、肺实质、淋巴管和血管中呈异常方式生长，导致肺囊性病变的发生。该疾病尚无治疗方法，会进展至呼吸衰竭，在此阶段肺移植（LT）可能是一种治疗选择。我们的目标是研究7例因S-LAM接受肺移植的患者。
我们研究了一系列临床和人口统计学特征、诊断方式及移植后结局。我们对该系列进行了描述性分析。采用Kaplan-Meier方法估计生存率。
症状出现的平均年龄为35岁，诊断时的平均年龄为37岁，接受肺移植时的平均年龄为38岁。最常见的症状是呼吸困难。4例患者有气胸和胸腔积液病史。一秒用力呼气量平均为32.7%，一氧化碳弥散量为29%。所有患者均接受了肺移植，1年、3年和5年的生存率分别为100%、85.7%和57.1%。3例死于闭塞性细支气管炎，2例尸检未显示疾病复发的证据。
对于呼吸功能严重受损的S-LAM患者，肺移植是一种治疗选择。
