淋巴管平滑肌瘤病

淋巴管平滑肌瘤病 (ｌｙｍｐｈａｎｇｉｏｌｅｉｏｍｙｏｍａｔｏｓｉｓ ,ＬＡＭ)是一种病因不明、持续发展的弥漫性肺间质疾病 ,主要发生在育龄期妇女。病理方面的特点为肺间质的平滑肌过度增生及肺囊肿的形成 ,其临床症状、Ｘ线表现、肺生理方面较为接近肺气肿的表现 ,存在气流受阻 ,而易误诊为哮喘或慢性阻塞性肺疾病。因患者有进行性呼吸困难及肺弥漫性阴影及隐约可见的囊肿 ,亦常误诊为特发性肺纤维化 (ＩＰＦ)。自 195 5年发现此病到 1981年搜集文献总报告例数 <10 0例。以往多认为其是一种罕见病。但我院自 1997年以来已先后确诊6例…     

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。     

肺淋巴管平滑肌瘤病的临床分析

目的探讨肺淋巴管平滑肌瘤病(LAM)的临床特征,治疗和预后。方法回顾性分析1例LAM病人并复习文献78例。结果全部79例患者中,女性占多数74例(93.7%)。发病年龄:69例(87.3%)为育龄期妇女,均经肺组织活检病理确诊。临床表现为呼吸困难(75例),咯血(40例),气胸(38例),乳糜胸(24例),行胸部高分辩CT(HRCT)扫描63例,均显示两肺弥漫性薄壁囊状阴影;行肺功能检查45例,阻塞性通气功能障碍32例,混合性通气功能障碍9例,行血气分析43例,其中低氧血症41例,型呼吸衰竭2例;给予安宫黄体酮及其他药物治疗32例,其中死亡或病情加重19例,其中11例从确诊到死亡时间为0.5-3.5年。结论LAM以呼吸困难,咯血、气胸和乳糜胸为主要临床特征,安宫黄体酮疗效不理想。     

肺淋巴管平滑肌瘤病10例临床分析

肺淋巴管平滑肌瘤病（PLAM）是一种罕见的肺部弥漫性疾病,主要以细支气管周围血管平滑肌异常增生为特征,伴气道或血管阻塞.目前病因未明,绝大部分发生于育龄期妇女,全球发病率约1/400 000.我们对我院收治的10例PLAM患者的临床资料及薄层CT（HRCT）表现进行分析,以提高对该病的认识,从而减少漏诊、误诊的发生.     

肺淋巴管平滑肌瘤病1例

肺淋巴管平滑肌瘤病（pulmonary lymphangioleiomyomatosis,PLAM）是一种少见疾病,多以肺部多发结节为表现,可以发生在双肺或者单肺,并以咳嗽、呼吸困难为首发症状,多发生于育龄妇女,男性罕见,常被误诊为肺部转移性肿瘤。PLAM症状多样,临床表现复杂,术前若无病理很难诊断,有时冰冻切片也不能确诊。     

肺淋巴管平滑肌瘤病一例

正肺淋巴管肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)是一种罕见的弥漫性肺部疾病,绝大多数发生于育龄期妇女,亦有男性患者报道。本疾病发病率大约为1/100万。表现为不典型增生的平滑肌细胞侵袭肺组织,以进行性呼吸困难、反复气胸及乳糜胸为主要临床表现,最终因呼吸衰竭死亡。由于罕见而散发,且临床表现多样,常被误诊,现将我科收治的1例PLAM患者的临床资料进行报道,以提高对该病的认识,减少误诊和漏诊。     

肺淋巴管平滑肌瘤病

淋巴管平滑肌瘤病是一种较为罕见的疾病,由于平滑肌样细胞异常增生导致肺囊肿的形成,导致肺功能进行性的下降,最终死亡。本病主要累及妇女,病因目前尚不清楚,亦无特异性治疗,本文对近来关于该病的报道作一综述。     

肺淋巴管平滑肌瘤病

目的 提高对罕见病淋巴管平滑肌瘤病（ＰＬＡＭ）的认为。方法 对１９９２年对来诊治的３例ＰＬＡＭ患者的临床资料进行分析,并结合文献复习。结果 ＰＬＡＭ是一种罕见的弥漫肺部疾病,临床表现为反复发作自发性气胸、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,动脉血气示低氧血症。胸部Ｘ线表现为两肺弥漫分布网格状影,高分辨ＣＴ（ＨＲＣＴ）示两肺弥漫分布囊状改变。病理学检查示肺组织淋巴管增生和扩张,     

肺淋巴管平滑肌瘤病

淋巴管平滑肌瘤病 (lymphangiolaiomyomatosic,LAM)是一种女性育龄期的少见病。自 1937～ 1997年60年间世界仅陆续报告了 30 0余例。由于罕见与散发 ,又缺少动物模型与组织培养不能深入研究。病因不明 ,亦缺少有效的治疗方法。病情进行性发展 ,最终死于慢性呼吸衰竭。提高对 LAM的诊断水平十分必要。我院两年间确诊两例 ,现结合文献进行讨论。例一　女性、4 0岁 ,1996年出现逐渐加重的呼吸困难与咳嗽 ,有少许白粘痰 ,时常痰中带血。一年后病情加重 ,胸片见弥漫性小结节、网纹增加 ,肺体积不缩小。CT见均匀分布大小不等的薄壁囊肿 ,并见…     

120例肺淋巴管平滑肌瘤病临床分析

目的通过分析中国大陆肺淋巴管平滑肌瘤病（PLAM）临床资料及误诊情况,以期提高医生对PLAM的认识。方法运用中国学术期刊全文数据库搜索截至2008年底由中国大陆学术期刊所发表的有关PLAM病例的文献,进而分析病例资料。结果120例入组患者确诊时的平均年龄是（37．26±6．40）岁。患者从出现症状到确诊平均需要（29．55±35．76）个月。该病患者临床表现多样,胸片无特征性改变。有10例患者合并肾血管平滑肌脂肪瘤,16例存在腹膜后肿块或淋巴结肿大。有64例患者在确诊前曾被误诊,易被误诊为特发性问质性肺炎或特发性气胸。结论需要进一步提高医生对PLAM的认识,以期减少误诊。     

Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex

The true prevalence of pulmonary lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex (TSC) is unknown. The prevalence of LAM, radiological features, and lung function in patients with TSC was measured. The presence of LAM, as defined by the presence of cysts by high-resolution chest computed tomography (HRCT) scan, was determined in patients with TSC without prior pulmonary disease (Group 1). To determine the significance of early detection, severity of disease in screened patients (Group 1) was compared with that in patients with TSC with a prior diagnosis of LAM (Group 2). Forty-eight patients with TSC and no prior history of LAM were screened. Of the 38 females, 13 (34%) had LAM; LAM was absent in males. Lung function was preserved in patients with TSC who were found to have LAM by screening. In patients previously known to have LAM, FEV(1) and DL(CO) correlated inversely with severity of disease as assessed by CT scan. The prevalence of LAM in women with TSC was 34%, approximately 10-fold that previously reported, consistent with a large hitherto unrecognized subclinical population of patients at risk for pulmonary complications.     

Basic fibroblast growth factor and its receptors in idiopathic pulmonary fibrosis and lymphangioleiomyomatosis

Basic fibroblast growth factor (bFGF) is a potent mitogenic factor for smooth muscle cells, myofibroblasts, and fibroblasts, proliferation of which is a hallmark of idiopathic pulmonary fibrosis (IPF) and lymphangioleiomyomatosis (LAM). Mast cells produce bFGF and have been associated with pulmonary fibrosis. We hypothesize that smooth muscle cell/myofibroblast-like cells will be spatially associated with bFGF-containing mast cells and that bFGF receptors will be expressed on the effector cells in IPF and LAM. We performed quantitative immunohistochemistry for bFGF, mast cell tryptase, smooth muscle actin for smooth muscle cell/myofibroblast-like cells, and fibroblast growth factor receptors (Flg, Bek) and measured collagen and elastic fiber in lung sections from IPF (n = 14), LAM (n = 9), and control lung (n = 10). IPF and LAM lung contained more smooth muscle cell/myofibroblast-like cells than did control lung. bFGF-containing mast cells were abundant both in IPF and LAM and were associated with collagen, elastic fibers, and smooth muscle cell/myofibroblast-like cells in IPF. Flg was expressed on epithelial cells, endothelial cells, smooth muscle cell/myofibroblast-like cells, and macrophages in IPF. In LAM, Flg was expressed on epithelial cells adjacent to smooth muscle cell/myofibroblast-like cell aggregates. Bek was expressed dominantly on smooth muscle cell/myofibroblast-like cells in LAM and on smooth muscle cell/myofibroblast-like cells as well as neutrophils in IPF. These data suggest that mast cell-derived bFGF might exert fibrogenic, proliferative effects on smooth muscle cell/myofibroblast-like cells through its receptors.     

Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients

This retrospective, multicentre study evaluated patients with lymphangioleiomyomatosis (LAM) and pre-capillary pulmonary hypertension (PH) by right heart catheterisation. It was conducted in 20 females with a mean±sd age of 49±12 yrs and a mean±sd time interval between LAM and PH diagnoses of 9.2±9.8 yrs. All, except for one patient, were receiving supplemental oxygen. 6-min walking distance was mean±sd 340±84 m. Haemodynamic characteristics were: mean pulmonary artery pressure (PAP) 32±6 mmHg, cardiac index 3.5±1.1 L·min(-1)·m(-2) and pulmonary vascular resistance (PVR) 376±184 dyn·s·cm(-5). Mean PAP was >35 mmHg in only 20% of cases. The forced expiratory volume in 1 s was 42±25%, carbon monoxide transfer factor was 29±13%, and arterial oxygen tension (P(a,O(2))) was 7.4±1.3 kPa in room air. Mean PAP and PVR did not correlate with P(a,O(2)). In six patients who received oral pulmonary arterial hypertension (PAH) therapy, the PAP decreased from 33±9 mmHg to 24±10 mmHg and the PVR decreased from 481±188 dyn·s·cm(-5) to 280±79 dyn·s·cm(-5). The overall probability of survival was 94% at 2 yrs. Pre-capillary PH of mild haemodynamic severity may occur in patients with LAM, even with mild pulmonary function impairment. PAH therapy might improve the haemodynamics in PH associated with LAM.     

Unilateral lymphangioleiomyomatosis

Pulmonary involvement in tuberous sclerosis complex (TSC) resembles lymphangioleiomyomatosis (LAM) in its clinical, radiologic, and histopathological features. Typical high-resolution computed tomography (HRCT) findings are of bilateral thin-walled cysts symmetrically throughout both lungs, with associated increase in lung volumes. Atypical cases have been described; and we report a case of biopsy proven, focal unilateral disease. The presence of nodules has been reported in association with TSC that histologically are composed of proliferated type II pneumocytes. We discuss other atypical cases of TSC and sporadic LAM and review current concepts of the pathogenesis of the 2 diseases, with reference to our case.