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。     

N端B型利钠肽原对急性肺血栓栓塞症患者预后评估价值

目的:探讨N端B型利钠肽原(NT-proBNP)对急性肺血栓栓塞症(PTE)患者的预后评估价值。方法:确诊为急性肺血栓栓塞症患者55例,以0~49岁NT-proBNP≥450 pg/ml,50~75岁NT-proBNP≥900 pg/ml,76~100岁NT-proBNP≥1 800 pg/ml为NT-proBNP(+)组(n=31),以0~49岁NT-proBNP450 pg/ml,50~75岁NT-proBNP900 pg/ml,76~100岁NT-proBNP1 800 pg/ml为NT-proBNP(-)组(n=24)。比较NT-proBNP(+)组和NT-proBNP(-)组间体循环收缩压、心率、血氧分压、右心室舒张末期前后直径、肺动脉高压发生率、临床不良事件发生率的差异性,同时比较有无临床不良事件发生的患者NT-proBNP水平的差异。结果:NT-proBNP(+)组较NT-proBNP(-)组心率增加显著(P0.05),体循环收缩压和氧分压下降显著(P0.05),差异均有统计学意义。超声心动图指标右心室舒张末期前后直径和肺动脉高压发生率均显著增加(P0.01),临床不良事件发生率显著增加(P0.01),差异均有统计学意义。共3例死亡,均发生在NT-proBNP(+)组。有临床不良事件发生的患者(15例)NT-proBNP中位数7 436 pg/mL(140~45 720 pg/mL)比无临床不良事件发生患者(40例)的NT-proBNP中位数812 pg/mL(32~13 529 pg/mL)水平明显升高(P0.01),差异有统计学意义,多元回归分析在校正了年龄、性别、高危PTE和中危PTE后,升高的NT-proBNP仍旧是发生临床不良事件的独立危险因素(比值比:15.3,95%可信限1.2~134.0,P0.05)。结论:高危和中危PTE患者多有血浆中NT-proBNP的水平升高,NT-proBNP的水平升高,提示预后不良。正常NT-proBNP的水平提示患者预后良好,NT-proBNP在PTE危险分层中起一定的作用。     

肺淋巴管平滑肌瘤病的诊治进展

<正>肺淋巴管肌瘤病(lymphangioleiomyomatosis,LAM)是以肺部广泛囊性病变为特征的一种罕见疾病,主要发生在育龄期女性,其发病的基本特征为肺部淋巴管平滑肌细胞(LAM细胞)异常增殖引起肺组织的损害和囊性重建[1,2]。一般为独立疾病,有时可与遗传病结节性硬化症(tuberous sclerosis complex,TSC)合并发生。LAM患者的临床症状主     

血管内皮生长因子-D表达与非小细胞肺癌淋巴管生成和淋巴结转移的相关性

目的观察血管内皮生长因子(VEGF-D)在非小细胞肺癌(NSCLC)组织中央部位、侵犯边缘和癌周肺组织的表达情况及肺癌组织微淋巴管密度(LMVD)与淋巴结转移、淋巴管侵犯和淋巴管生成的相关性。方法选择人NSCLC新鲜组织样本96例,应用免疫组织化学法和实时适量(QRT)-PCR法检测NSCLC组织中央部位、侵犯边缘和癌周肺组织的VEGF-D蛋白及mRNA表达情况,应用单克隆抗体(D2-40)标记淋巴管,检测NSCLC组织中的LWVD。结果 QRT-PCR显示,VEGF-D mRNA在肿瘤边缘部位的表达明显高于在肿瘤中央部位的表达,而且VEGF-D mRNA的表达亦高于其在周围肺组织的表达。免疫组织结果显示了同样的表达不均一性。肿瘤边缘VEGF-D高表达组淋巴结转移的发生率明显高于低表达组,而且VEGF-D高表达组其淋巴管侵犯的发生率亦明显增高。肿瘤边缘高LWVD组的VEGF-D mRNA的表达也明显高于低密度组。结论肿瘤侵犯边缘的VEGF-D的高表达与淋巴结转移和淋巴管侵犯密切相关,参与了NSCLC的淋巴管生成和淋巴结转移过程。     

肺淋巴管平滑肌瘤病肺移植治疗研究进展

肺淋巴管平滑肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)是一种罕见的、发病机制尚不清楚的弥漫性肺部疾病,常见于育龄期妇女,与结节性硬化症(tuberous sclerosis,TSC)基因1和2的突变有关[1,3].该病以异常的平滑肌细胞(LAM细胞)在气道、淋巴管及血管等中异常增殖为特点,最终导致肺实质形成薄壁囊状结构,肺功能减退甚至呼吸衰竭,从而危及患者生命.内科治疗效果欠佳,肺移植为目前惟一可能有效的治疗手段.     

心力衰竭患者B型利钠肽的测定与评价

目的:研究B型利钠肽(BNP)在心力衰竭诊断中的应用。方法:采用双抗夹心免疫荧光法检测342例患者的BNP浓度。其中充血性心衰(CHF,心源性)组179例,肺源性心衰组27例,无心衰的对照组136例。结果:(1)在无心衰对照组中,年龄小于55岁组的心源性浓度均值为(35.10±31.93)pg/ml,55～64岁组为(42.85±34.55)pg/ml,65～74岁组为(58.23±25.37)pg/ml,≥75岁组为(60.66±48.95)pg/ml,各组间BNP浓度均值虽随年龄呈上升趋势但无显著差异;(2)对照组中男性BNP浓度为(42.83±38.01)pg/ml,女性为(49.47±48.14)pg/ml,两者无显著差异;(3)在342例患者中BNP以100pg/ml为CHF的判断值时,其敏感性达92.8%,特异性达83.2%,阳性预测值达86.2%,阴性预测值达91.2%;(4)CHF(心源性)患者的BNP浓度均值为(1019.13±972.88)pg/ml,肺源性心衰组为(299.88±275.5)pg/ml,对照组为(45.86±42.89)pg/ml,CHF(心源性)组BNP水平显著高于其他两组,肺源性心衰组又显著高于对照组(P均<0.001)。结论:BNP测定不仅可以作为CHF诊断的一个重要指标,还可用于参考性区分心源性心衰和肺源性心衰,为选择治疗方案提供依据。     

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2例并文献复习

目的探讨肺淋巴管平滑肌瘤病的临床特征、治疗和预后,提高对该病的认识。方法回顾性分析2例肺淋巴管平滑肌瘤病患者的临床资料并结合文献复习。结果该病主要发生在育龄期妇女,临床主要表现为渐进性呼吸困难、反复发作性气胸、乳糜性胸腔积液等。HRCT以肺部均匀分布的多个薄壁囊状影为特征,肺组织活检是诊断金标准,治疗无特效方法,主要是药物及肺移植。结论肺淋巴管平滑肌瘤病罕见,临床工作中若遇育龄期妇女出现渐进性呼吸困难、反复发作性气胸、乳糜性胸腔积液等应考虑有无肺淋巴管平滑肌瘤病可能,及时行HRCT检查,必要时肺组织活检明确诊断。     

淋巴管平滑肌瘤病合并乳糜肺炎一例

淋巴管平滑肌瘤病(LAM)是一种少见的系统性低度恶性肿瘤性疾病。LAM合并乳糜肺炎较为少见,易误诊、漏诊。本文报道1例LAM合并乳糜肺炎,患者胸部CT示双肺散在小圆形囊状透亮影、斑片影、磨玻璃影。血清血管内皮生长因子D(VEGF-D)11370.2 ng/L,痰乳糜试验阳性。腹部CT示腹膜后可见多发低密度团块影。予西罗莫司维持治疗(血药浓度5~10μg/L),随访18个月,患者症状改善,肺部病变、腹部病变逐渐吸收,血清VEGF-D水平降至3231.9 ng/L。胸部CT示肺部出现小叶间隔增厚,磨玻璃影、斑片影等影像学表现时,需考虑乳糜肺炎可能;VEGF-D对该病的诊断和疗效评估具有重要意义;西罗莫司对LAM合并乳糜肺炎疗效显著,但其长期使用的有效性、安全性需进一步探究。     

Micronodular pneumocyte hyperplasia in pulmonary lymphangioleiomyomatosis and tuberous sclerosis

An open lung biopsy was performed in a 28-year-old woman with tuberous sclerosis and with spontaneous pneumothorax and interstitial changes in the chest X-ray. Microscopically a micronodular pneumocyte hyperplasia in addition to a lymphangioleiomyomatosis was found. The micronodular pneumocyte hyperplasia is considered to be a very rare lesion. Only 27 well documented such cases have been recorded so far, 24 in women and 3 in men. The age of the patients ranged between 20 and 57 years, with an average of 36 years. The micronodular pneumocyte hyperplasia occurs mainly in patients with tuberous sclerosis (20 of 27 cases) and it is often combined with a lymphangioleiomyomatosis (19 cases). In the differential diagnosis the atypical adenomatous hyperplasia is to be considered in the first place. In contrast to it, the proliferating cells of the micronodular pneumocyte hyperplasia show no atypia. The differentiation from a papillary adenoma can be difficult in small biopsies.     

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.     

Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis

OBJECTIVES: To evaluate comprehensively the characteristics of lymphangioleiomyomatosis (LAM), with emphasis on the application of imaging and immunohistochemical methods. DESIGN: Prospective study. PATIENTS: Thirty-five female subjects with LAM. SETTING: Clinical Center, National Institutes of Health. INTERVENTIONS: BAL, pulmonary function test, ventilation/perfusion lung scans, CT of the chest and abdomen, ultrasonography of abdomen, and immunohistochemical study of lung biopsy specimens. RESULTS: Most patients had exertional dyspnea (83%) and pneumothorax (69%). BAL did not show diagnostic changes. The most common abnormalities on pulmonary function tests were decreased diffusing capacity of carbon monoxide (83%), hypoxemia (57%), and airway obstruction (51%). Bronchodilator response was found in 26% of patients. CT, which is almost pathognomonic, showed numerous thin-walled cysts throughout both lungs in all patients. Thirty-four patients (97%) had abnormal ventilation and/or perfusion lung scans. An unusual "speckling" pattern was observed on ventilation scans of 74% of patients. Common extrapulmonary features were retroperitoneal adenopathy (77%) and renal angiomyolipomas (60%). The percentage of abnormal smooth muscle cells (LAM cells), reactive with HMB45, varied from 17 to 67% in 10 lung biopsy specimens. CONCLUSIONS: Improved diagnostic methods have defined the abnormalities in patients with pulmonary LAM and increased the potential for early recognition and treatment of this disorder. Patients with LAM should be evaluated for bronchodilator responsiveness and may benefit from a trial of bronchodilators.     

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.     

Lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare disease of the lungs and lymphatics, which can occur sporadically or in association with tuberous sclerosis. LAM almost exclusively affects females, generally developing before the menopause. The disease is characterised by progressive pulmonary cystic change, recurrent pneumothorax, chylous pleural collections and, in most cases, progressive respiratory failure. Abdominal manifestations include lymphadenopathy, cystic lymphatic masses (lymphangioleiomyomas), chylous ascites and angiomyolipoma (a benign tumour). Survival in LAM is approximately 70% at 10 yrs, although this is highly variable since long-term survivors have been described. Diagnosis is made by a combination of clinical features and computed tomography scanning or, in cases of doubt, lung biopsy. In patients with rapidly progressive disease, hormone treatment (predominantly progesterone) has been used, although no firm evidence supports its use. Otherwise, treatment is aimed at complications including pneumothorax, chylous collections and extrapulmonary manifestations. The only treatment for severe LAM is currently lung transplantation. Recent developments in the cell biology of lymphangioleiomyomatosis have shown that these patients have somatic mutations in the genes linked to tuberous sclerosis and that rapamycin may correct the resulting cellular abnormality. Trials of rapamycin in lymphangioleiomyomatosis are currently underway and offer hope of evidence-based treatment for the disease.     

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。     

A case of pulmonary lymphangioleiomyomatosis originally treated as bronchial asthma]

The subject was a 37-year-old woman in whom bronchial asthma had been diagnosed, for which inhaled steroid and theophylline had been prescribed for about nine months. Her symptoms improved temporarily, but there-after she experienced gradually worsening dyspnea on exertion. Chest radiographs showed diffuse reticulonodular shadows, but no evidence of hyperinflation or pleural effusion. Chest CT scans revealed multiple thinwalled cystic lesions in both lung fields. Pulmonary lymphangioleiomyomatosis was confirmed by thoracoscopic lung biopsy, and medroxyprogesterone was prescribed. Her airways appeared to demonstrate hyperresponsiveness to inhaled methacholine and her symptoms resembled those of bronchial asthma. The relationship between pulmonary lymphangioleiomyomatosis and airway hyperresponsiveness should be considered in future.