摘要
目的提高对肺淋巴管平滑肌瘤病(PLAM)的认识和诊断水平.方法报道确诊为PLAM的新病例3例,结合1994年至2004年国内报道的PLAM病例共48例,对该病的临床特点进行汇总分析.结果 3例患者均为育龄女性患者,首发症状为活动后呼吸困难,其中2例合并肺外淋巴管平滑肌瘤,3例均无乳糜胸水.文献报道的48例PLAM患者中,47例有病理学诊断依据;发病年龄为5～69(平均34±10)岁,常见临床表现依次为呼吸困难(95.8%)、咯血(52.1%)、气胸(45.8%)、乳糜胸(33.3%)、咳嗽咳痰(31.3%)和胸痛(12.5%);16例发现腹部异常,包括肾内肿物、腹膜后肿物和腹膜后淋巴结肿大.39例行胸部高分辨CT检查,38例可见双肺弥漫分布的薄壁小囊影.30例有肺功能测定数据或结论的患者中,23例为阻塞性通气功能障碍,7例为混合性通气功能障碍.28例患者有动脉血气分析数据,其中17例合并呼吸衰竭.结论胸部高分辨CT对PLAM具诊断价值.对育龄妇女发生的渐进性呼吸困难、咯血、自发性气胸应及时进行胸部高分辨CT检查.并应常规进行腹部和盆腔影像学检查以了解患者是否合并肺外淋巴管平滑肌瘤.
Objective To improve the understanding and diagnosis of pulmonary lymphangioleiomyomatosis (PLAM) by the comprehensive review of domestic literatures in the past ten years.Methods Three new cases with PLAM were reported and integraed with other 45 cases reported domestically in the past ten years for analysis of their clinical features.Results The newly reported three cases of PLAM were all women at child-bearing age, with initial symptom of dyspnea after activity. Two of them complicated with extra-pulmonary PLAM. All the three cases were free of chylous effusion. Forty-seven of 48 cases with PLAM were pathologically diagnozed, with ages of onset of 5~69 (mean±s of 34±10) years. Their clinical manifestations were mainly respiratory, including dyspnoea (95.8%), haemoptysis (52.1%), pneumothorax (45.8%), chylous effusion (33.3%),cough (31.3%) and chest pain (12.5%). Abnormal manifestations in abdomen, including renal mass, retroperitoneal mass and retroperitoneal lymphadenopathy, were detected in 16 cases. Thirty-nine cases had their high-resolution CT (HRCT) examined and appearance of multiple cysts distributed throughout the bilateral lung fields could be discerned in 38 of them. Obstructive ventilation disturbance could be observed in 23 of 30 cases with the data or conclusions on pulmonary function tests, and mixed ventilation disturbance in seven cases. Respiratory failure was complicated in 17 of 28 cases with the data of arterial blood gas analyses.Conclusions HRCT had confirmative value for diagnosis of PLAM. In practice, HRCT, as well as other routine abdominal and pelvic imaging examinations, should be performed in time for child-bearing-age women with progressive dyspnoea, haemoptysis, or spontaneous pneumothorax, to detect if they complicate with PLAM.
出处
《中华全科医师杂志》
2005年第3期162-166,i001,共6页
Chinese Journal of General Practitioners
