肺淋巴管肌瘤病9例临床分析

目的探讨肺淋巴管肌瘤(PLAM)的临床表现及诊治方法。方法报道并临床分析2005—2008年中南大学湘雅二院收治的具有较完整资料的9例肺淋巴管肌瘤病例,结合文献进行复习。结果 9例均为女性。均有活动后气促,1例有乳糜腹,2例有乳糜胸,3例有气胸,6例有咯血。9例高分辨CT(HRCT)均示肺部弥漫分布薄壁囊腔透光区的PLAM特征性表现而临床诊断。4例行病理组织检查确诊。国内首次报道予雷帕霉素治疗LAM且疗效满意的病例。结论 HRCT检查对PLAM具诊断价值,对育龄妇女发生的渐进性呼吸困难、气胸、乳糜胸、咯血应及时进行胸部HRCT检查,雷帕霉素给此病的治疗带来新希望。     

肺淋巴管肌瘤病一例临床报告及文献复习

目的提高对肺淋巴管平滑肌瘤病(pulmonary lymphangidei omyomatosis,PLAM)诊断和难治性气胸的认识。方法报道确诊为PLAM的新病例1例,结合国内报道的PLAM病例,对该病的临床特点进行汇总分析。结果肺淋巴管肌瘤病易并发难治性气胸,50%以此为首发症状,80%在疾病过程中发生气胸。气胸常常表现为复发性,双测性。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。     

肺淋巴管肌瘤病11例报告并文献复习

目的 探讨肺淋巴管肌瘤病( PLAM)的临床和预后.方法 分析经治的11例PLAM病人的资料,并复习文献.结果 11例均为女性;年龄31 ～55岁,平均(44±9)岁;均经肺组织活检明确病理;主要临床表现为进行性呼吸困难10例(发生率91％),咳嗽6例(55％),咯血2例(18％),乳糜胸3例(27％),气胸3例(27％)等;11例患者均进行肺功能检测,均有弥散功能下降,9例行动脉血气分析,其中7例表现为低氧血症,4例合并呼吸衰竭;11例行胸部高分辨率CT (HRCT),均显示两肺弥漫性薄壁囊状阴影.结论 PLAM以呼吸困难,咳嗽,气胸和乳糜胸为主要临床表现;双肺弥漫性薄壁囊状阴影为影像学特征表现;肺组织活检是确诊的主要依据.     

肺淋巴管平滑肌瘤病二例―附文献分析

目的提高对肺淋巴管平滑肌瘤病(PLAM)的认识并引起对该病的重视.方法对2例PLAM患者的临床资料进行分析,并结合文献进行复习.结果PLAM是一种持续发展的弥漫性肺间质疾病.临床表现为反复发作的自发性气胸、活动后呼吸困难、咯血和乳糜胸等.肺功能呈阻塞性肺通气功能障碍和弥散功能障碍,动脉血气分析显示低氧血症.胸部X线表现两肺弥漫性网格状,晚期呈蜂窝样变化.高分辨CT(HRCT)两肺广泛弥漫性分布的薄壁小囊状病变.病理学特点为肺淋巴管、小气道、小血管的管壁及其周围的平滑肌细胞弥漫性异常增生.免疫组化HMB45阳性.结论对育龄期妇女发生渐进性呼吸困难不能缓解,类似肺气肿的临床表现及X线胸片阴影,并出现反复气胸或乳糜胸水时即应考虑为PLAM可能,最好能以肺组织活检明确诊断.该病预后差,目前无有效的预防和治疗方法.     

西罗莫司治疗肺淋巴管肌瘤病伴巨大腹膜后淋巴管肌瘤1例及文献复习

<正>淋巴管肌瘤病 (lymphangioleiomyomatosis, LAM)是一种以育龄期女性出现肺部弥漫性囊性病变为主要特征的罕见疾病，常见临床表现为进行性呼吸困难、反复发作的气胸及乳糜胸，肺外表现以肾血管平滑肌脂肪瘤(angiomyolipoma, AML) 多见，目前国内报道LAM合并巨大腹膜后淋巴管平滑肌瘤罕见。本文总结1例西罗莫司成功治疗肺淋巴管肌瘤病伴巨大腹膜后淋巴管肌瘤，并结合文献进行复习，提高临床医生对该疾病的认识。     

肺淋巴管平滑肌瘤病1例并文献复习

目的探讨肺淋巴管平滑肌瘤病（PLAM）的临床特征、疗效和预后,提高对该病的认识。方法回顾性分析1例PLAM病人的临床资料,并复习文献。结果该病主要发生于育龄期妇女,临床症状无特异性,以咳嗽、胸闷、气短和呼吸困难为主;常发生乳糜胸和（或）气胸;肺功能检查有弥散功能下降,以阻塞性通气功能障碍为多,兼有限制性通气功能障碍;HRCT见双肺弥漫薄壁囊状阴影为影像学特征性表现;肺组织活检是确诊的主要依据。结论本病病因目前尚不清,孕激素疗效不理想,有待进一步探讨病因及寻找有效的疗法。     

肺淋巴管平滑肌瘤病二例一附文献分析

目的 提高对肺淋巴管平滑肌瘤病(PLAM)的认识并引起对该病的重视。方法 对2例PLAM患者的临床资料进行分析，并结合文献进行复习。结果 PLAM是一种持续发展的弥漫性肺间质疾病。临床表现为反复发作的自发性气胸、活动后呼吸困难、咯血和乳糜胸等。肺功能呈阻塞性肺通气功能障碍和弥散功能障碍，动脉血气分析显示低氧血症。胸部x线表现两肺弥漫性网格状，晚期呈蜂窝样变化。高分辨CT(HRCT)两肺广泛弥漫性分布的薄壁小囊状病变。病理学特点为肺淋巴管、小气道、小血管的管壁及其周围的平滑肌细胞弥漫性异常增生。免疫组化HMB45阳性。结论 对育龄期妇女发生渐进性呼吸困难不能缓解，类似肺气肿的临床表现及x线胸片阴影，并出现反复气胸或乳糜胸水时即应考虑为PLAM可能，最好能以肺组织活检明确诊断。该病预后差，目前无有效的预防和治疗方法。     

淋巴管平滑肌瘤病的研究进展

淋巴管平滑肌瘤病(lymphangioleiomyomatosis,LAM)是一种主要影响育龄期女性的罕见疾病。该病为多系统疾病,主要累及肺脏,表现为两肺弥漫性囊泡改变,常合并肾血管平滑肌脂肪瘤(angiomyolipoma,AML)、后腹膜、盆腔淋巴结肿大等肺外表现。临床上主要表现为进行性加重的呼吸困难、反复自发性气胸、顽固性乳糜胸等。该病的发生     

肺淋巴管肌瘤病的临床及病理特点

目的 提高对肺淋巴管肌瘤病(lymphangioleiomyomatosis,LAM)的临床及病理诊断和认识水平.方法 回顾分析复旦大学附属中山医院确诊为肺LAM的病例15例,并复习1993-2008年国内文献的73例报道,分析该病的临床及病理特点.其中1例患者死后行尸体解剖,对其全身各脏器进行病理组织学观察.结果 88例患者均为女性,平均发病年龄(37±9)岁,常见临床表现依次为呼吸困难83例(94%)、咯血48例(54%)、气胸41例(47%)、乳糜胸28例(32%).其中35例患者行肺功能检查,32例存在弥散功能障碍,25例表现为阻寨性通气功能障碍,10例表现为混合性通气功能障碍.胸部高分辨率CT检查显示双肺为典型的弥漫性薄壁囊状阴影.病理检查结果显示肺组织旱弥漫性囊状改变,增生的LAM细胞沿细支气管壁、肺泡壁、淋巴管肇和血管壁周围分布,形成结节状.1例尸解病理检查结果显示肺脏、肾脏、淋巴结及软组织等多器官受累.42例行腹部B超及影像学检查,其中23例伴发肺外LAM.结论 LAM常累及全身多个系统,但肺是最主要的受累器官.育龄期妇女出现进行性呼吸困难、气胸、乳糜胸及高分辨CT表现为弥漫性小囊状改变时,应考虑到LAM的可能.确诊需依赖肺组织活检病理检查结果,并应常规进行腹部及盆腔影像学检查,以了解患者是否合并肺外LAM.     

肺淋巴管平滑肌瘤病九例临床和病理分析

目的 探讨肺淋巴管平滑肌瘤病(pulmonary lymphangionleiomyomatosis,PLAM)的临床和病理特征,提高临床诊治水平.方法 对广州呼吸疾病研究所确诊的9例PLAM患者的临床特点、影像学表现、肺功能及病理学检查进行分析并结合文献复习.结果 9例PLAM患者均为育龄期妇女(20～50岁),临床症状表现为呼吸困难(8/9)、咳嗽(6/9)、咯痰(2/9)、咯血(2/9)、胸痛(3/9)、自发性气胸(6/9)、乳糜胸(3/9)、乳糜性腹腔积液(2/9).胸部X线检查4例表现为双肺弥漫性网状结节影,2例表现为液气胸,2例表现为胸腔积液,1例表现为多发肺小囊状透光区及肺大疱,1例无异常.高分辨率CT具有特征性变化,9例双肺均可见多发小囊状影,直径2～20 mm,壁较薄.2例腹部CT于腹主动脉前可见多个肿大的淋巴结.6例患者进行了肺功能检查,3例通气功能正常,1例呈中度阻塞性通气功能障碍,1例呈重度混合性通气功能障碍,1例呈极重度混合性通气功能障碍,其中1例支气管舒张试验阳性.4例肺弥散功能下降.4例(4/9)经纤维支气管镜肺活检,5例(5/9)经胸腔镜活检,全部经病理确诊.病理学检查显示不同成熟度平滑肌细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生,肺实质呈囊性变.结论 PLAM表现不典型,易被漏诊、误诊,应提高对本病的认识.     

肺淋巴管平滑肌瘤病

目的　提高对罕见病肺淋巴管平滑肌瘤病 (PL AM)的认识。方法　对我院诊治的患者进行分析 ,并结合文献复习。结果　 PL AM是一种罕见的弥漫性肺部疾病 ,临床反复发作自发性气胸或 /和乳糜胸、活动后呼吸困难和痰血等。肺功能呈阻塞性或混合性通气功能障碍 ,低氧血症。胸部 X线表现为两肺弥漫分布网格状阴影 ,高分辨 CT(HRCT)示弥漫分布囊状改变。病理学检查示 :肺组织淋巴管增生和扩张 ,管外平滑肌明显增生。结论　育龄期妇女如反复发生气胸及出现原因不明的呼吸困难、咯血或乳糜胸 ,应疑诊 PL AM,及时行 HRCT及肺功能检查 ,必要时肺活检 ,以明确病理诊断。     

The association of pulmonary lymphangioleiomyomatosis with renal and hepatic angiomyolipomas in a prepubertal girl: a previously unreported entity

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare, diffuse progressive interstitial lung disease that affects females of childbearing age and is characterized by diffuse proliferation of abnormal smooth muscle fibers predominantly developing in the lung and leading to cystic destruction. A prepubertal 13-year-old girl with PLAM associated with renal and hepatic angiomyolipomas who was treated by nephroureterectomy and thoracoscopic pleurodesis is presented. To the best of our knowledge, this is the first reported case of PLAM associated with renal and hepatic angiomyolipomas at the prepubertal age. After evaluating the clinicopathologic features of this rare entity, the authors conclude that PLAM should be considered in the differential diagnosis of cystic pulmonary pathologies in children, particularly teenagers. The most important clinical aid is to bear this rare entity in mind when a child presents with renal and/or hepatic angiomyolipomas. Thoracoscopic pleurodesis is the most effective treatment modality for recurrent pneumothoraces.     

When to suspect and how to diagnose pulmonary lymphangioleiomyomatosis

Abstract The objective of this study was to present clinical and radiological data of eight women with histologically proven lymphangioleiomyomatosis (LAM) diagnosed between 1984 and 1994, and to suggest a diagnostic strategy when LAM is suspected. A review of case reports, including results of biopsies, lung function and radiological procedures was undertaken. The mean age of the women at start of symptoms was 36 years, and the mean age at time of diagnosis 42 years. The most frequent presenting complaint was dyspnea, either in conjunction with pneumothorax (3), chylothorax (2) or on exertion (2). All patients had airflow limitation and markedly reduced gas transfer. Five patients had 16 episodes of pneumothorax. In seven patients multiple cysts were observed on the surface of the lung during thoracotomy while computerized tomography (CT) scans revealed numerous cysts evenly distributed throughout the lung parenchyma. The procedures that confirmed the diagnosis included transbronchial lung biopsy (4), open lung biopsy (2), thoracoscopy (1), thoracotomy (3) and autopsy (1). Three specimens had to be revised before the histological diagnosis was confirmed. It was concluded that the important clues to a diagnosis of LAM are recurrent episodes of pneumothoraces in fertile women, progressive air-flow limitation, markedly reduced gas transfer and characteristic findings on thoracic CT scans. A specific request to the pathologist to stain lung tissue specimens for smooth muscle cells is mandatory.     

Rare pulmonary manifestations of tuberous sclerosis in children

Tuberous sclerosis is one of the rare neurocutaneous syndromes and may involve organs other than skin and brain. Pulmonary involvement is uncommon and predominantly affects women of childbearing age. Those affected present with dyspnea or spontaneous pneumothorax from cystic or fibrous degeneration of the lung. Males are affected very rarely. This paper describes another manifestation of pulmonary involvement in tuberous sclerosis, namely, recurrent intrapleural cysts in a young boy. Pediatr. Pulmonol. 1997; 23:114–116. © 1997 Wiley-Liss, Inc.