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目的:探讨肺淋巴管平滑肌瘤病(PLAM)的临床表现、肺功能、血气分析、影像学特点、病理特征及疗效和预后,提高对该病的认识和诊断水平,减少漏诊、误诊。方法:回顾确诊的2例肺淋巴管平滑肌瘤病例,并结合国内文献,对其进行汇总分析。结果:肺淋巴管平滑肌瘤病中,女、男发病比例为16.8∶1,发病年龄5～69(36.9±8.1)岁,其中育龄期女性占94%;主要临床表现呼吸困难发生率为80.4%,咳嗽咳痰为44.9%,气胸为32.7%,咯血为30.0%,乳糜胸、腹水为25.2%,胸痛为12.1%;肺功能测定数据中DLCO平均值为46.2±20.0,FEV1/FVC平均值为57.8±18.8,呈弥散功能障碍为84.5%,阻塞性通气功能障碍为70.7%,限制性通气功能障碍为18.9%,混合性通气功能障碍为22.4%;胸部X线表现为两肺弥漫分布网格状影,胸部CT见两肺分布小透亮影,胸部高分辨CT(HRCT)显示两肺弥漫分布囊状改变;动脉血氧分压(PaO2)平均值为(62.2±12.8)mmHg;支气管镜肺活检成功率达95.1%;免疫组化显示actin100%、HMB-4593.5%呈阳性表达;患者首次就诊时均被误诊;疗效欠佳,预后不理想。结论:呼吸困难、咳嗽咳痰、咯血、气胸和乳糜胸是PLAM的主要临床特征,主要侵袭生育期妇女,HRCT对PLAM有诊断意义,最好行肺活检(TBLB)明确诊断。
Objective: To investigate the clinical manifestations, pulmonary function, blood gas analysis, imaging features, pathological features, curative effect and prognosis of pulmonary lymphangioleiomyomatosis (PLAM), improve the level of understanding and diagnosis of PLAM, and reduce the missed diagnosis and misdiagnosis. Methods: Two cases of pulmonary lymphatic leiomyoma confirmed by diagnosis were retrospectively analyzed and analyzed according to the domestic literatures. Results: The incidence of pulmonary lymphangioleiomyomatosis was 16.8:1. The age of onset was 5-69 years (36.9 ± 8.1) years, of which 94% were women of reproductive age. The main clinical manifestations of dyspnea were 80.4 %, Cough and expectoration was 44.9%, pneumothorax was 32.7%, hemoptysis was 30.0%, chylothorax, ascites was 25.2%, chest pain was 12.1%; pulmonary function test data DLCO average of 46.2 ± 20.0, the average FEV1 / FVC 57.8 ± 18.8, diffuse dysfunction was 84.5%, obstructive ventilatory dysfunction was 70.7%, restrictive ventilatory dysfunction was 18.9%, mixed ventilatory dysfunction was 22.4%; chest X-ray showed diffuse distribution network of both lungs Chest CT showed small translucent shadow distribution of both lungs, high resolution chest CT (HRCT) showed diffusely distributed saccular changes in both lungs; arterial partial pressure of oxygen (PaO2) mean (62.2 ± 12.8) mmHg; bronchoscopy The success rate of lung biopsy was 95.1%. Immunohistochemistry showed that actin100% and HMB-4593.5% were positive. Patients were misdiagnosed on first visit. The curative effect was poor and the prognosis was not satisfactory. Conclusion: Dyspnea, cough and expectoration, hemoptysis, pneumothorax and chylothorax are the main clinical features of PLAM. They mainly affect women of reproductive age. HRCT has diagnostic value for PLAM, and is best diagnosed by lung biopsy (TBLB).