Nodular lung disease with five year survival and unilateral pleural effusion in AL amyloidosis.

A 67-year-old female patient with biopsy proven AL systemic amyloidosis developed rapidly progressive dyspnea. Chest roentgenogram and CT scan revealed a large right pleural effusion in addition to nodular lesions with bilateral hilar lymphadenopathy. The patient's serum showed IgG lambda type monoclonal gammopathy and she also had Bence Jones proteinuria. The pleural effusion was an exudate that contained many mononuclear cells and a high concentration of protein. Cardiac function was not seriously disturbed. Except for amyloidosis, no other causes for the severe pleural effusion were found. This patient was treated with chemical pleurodesis using Picibanil and a low dose of prednisolone. Eighteen months after this treatment, her right pleural effusion did not recur. Bronchopulmonary tissues are known to be frequently involved by AL systemic amyloidosis, but a nodular pattern of pulmonary amyloid deposition and a unilateral large pleural effusion are rare clinical manifestations in this disease.     

Pleural amyloidosis in a patient with intractable pleural effusion and multiple myeloma

Pleural involvement of systemic amyloidosis has been rarely reported. We report a case with multiple myeloma presenting an intractable right pleural effusion, in which pleural amyloidosis was diagnosed through pleural biopsy using a Cope needle. The diagnosis of pleural amyloidosis is important, because its refractory pleural effusion should be treated with pleurodesis. Since closed pleural biopsy using a Cope needle is much less invasive than thoracoscopy, the former should be attempted first whenever pleural amyloidosis is suspected.     

Interstitial lung disease with pleural effusion caused by Simvastin

De Groot REB, Willems LNA, Dijkman JH (Department of Pulmonology, Leiden University Hospital, Leiden, The Netherlands). Interstitial lung disease with pleural effusion caused by Simvastin (Case Report). J. Intern Med 1996; 239: 361–3.
Simvastin, a HMG-CoA reductase blocker, is used for the treatment of certain forms of hypercholesterolaemia. Simvastin is prescribed to lower high serum levels of cholesterol by inhibiting a specific enzyme, hydroxy-methylglutarylCo-enzym-A (HMG-CoA) reductase. This ultimately leads to an increase of the number of LDL-receptors in the liver, and thus, to a decrease of the serum LDL-cholesterol. To a much lesser extent it lowers the serum VLDL-cholesterol and makes the serum HDL-cholesterol level rise. In general, this relatively new compound is well tolerated and only a few, mostly minor, adverse effects have been reported so far. We present a patient who developed interstitial lung disease with pleural effusion most probably as a result of the use of Simvastin.     

Marked shrinkage of amyloid lymphadenopathy after an intensive chemotherapy in a patient with IgM-associated AL amyloidosis

A male patient with primary AL amyloidosis who had been suffering from systemic lymphadenopathy with IgMκ-type M-proteinemia received two courses of VAD and high-dose melphalan with in vivo elimination of CD20⁺ cells using rituximab followed by autologous peripheral blood stem cell transplantation. Four years after complete hematological remission he showed marked reduction in size of the amyloid-laden lymph nodes. Deposits of AL amyloid may regress from the tissue if the chemotherapy succeeds in persistent inhibition of the production of amyloidogenic immunoglobulin light chains.     

肺栓塞并胸腔积液的相关临床分析

目的探讨肺栓塞(Pulmonary Embolism,PE)并胸腔积液患者的临床特点。方法对确诊的115例肺栓塞患者,根据患者是否存在胸腔积液分为肺栓塞并胸腔积液组(实验组)及肺栓塞无胸腔积液组(对照组),比较两组的临床特点。结果合并胸腔积液有52例,无胸腔积液有63例。肺栓塞并胸腔积液患者的发热比率、呼吸次数、PT、APTT、FIB、INR、DD、HSCRP、cTnT、NTproBNP、RAD及mPAP高于无胸腔积液患者,差异有统计学意义(P<0.05);肺栓塞并胸腔积液患者的LYMPH、AT水平低于无胸腔积液患者,差异有统计学意义(P<0.05);经多因素logistic回归分析,FIB、DD、RAD及AT可能是PE发生胸腔积液独立预测因子,FIB、DD及RAD可能是PE患者发生胸腔积液的危险因素,AT可能为PE发生胸腔积液的保护因素。52例肺栓塞合并胸腔积液患者中,双侧胸腔积液33例(63.5%),单侧胸腔积液19例(36.5%);少量胸腔积液44例(84.6%),中-大量胸腔积液8例(15.4%);双侧少量胸腔积液29例(55.77%)。肺栓塞栓塞部位与是否发生胸腔积液比较,差异无统计学意义(P>0.05)。在肺栓塞并胸腔积液组中,肺栓塞栓塞部位与胸腔积液部位比较,差异无统计学意义(P>0.05);肺栓塞栓塞部位与胸腔积液量比较,差异无统计学意义(P>0.05);胸腔积液部位与胸腔积液量比较,差异无统计学意义(P>0.05)。肺栓塞并胸腔积液组不良事件发生率高于肺栓塞无胸腔积液组,差异有统计学意义(P<0.05)。结论FIB、DD、RAD及AT可能是PE发生胸腔积液的影响因素。肺栓塞并胸腔积液多为双侧少量胸腔积液。肺栓塞部位、胸腔积液量及胸腔积液部位之间未发现有相关性。胸腔积液对PE患者短期预后的评估具有一定价值。     

Association of acquired von Willebrand syndrome with AL amyloidosis

Acquired loss of functional von Willebrand factor (VWF) has been termed the acquired von Willebrand syndrome (AVWS). AVWS is a rare adult-onset bleeding diathesis that is clinically similar to congenital von Willebrand disease (VWD), and occurs with a variety of autoimmune, lymphoproliferative, or myeloproliferative disorders. We have identified four patients with AVWS in association with immunoglobulin light chain (AL) amyloidosis. These patients, lacking any pre-existing or family history of abnormal bleeding, developed cutaneous, mucosal, or gastrointestinal bleeding in the course of their disease without deficiency of clotting factor X or other factors; the activated partial thromboplastin time (aPTT) was prolonged in three out of the four cases. Despite normal VWF antigen levels, VWF ristocetin cofactor activity (VWF:RCo) was low. Electrophoresis patterns of high molecular weight (HMW) VWF multimers were abnormal in two of the four cases. Two of the patients were treated with high-dose intravenous melphalan followed by autologous stem cell transplantation (HDM/SCT) and achieved hematologic remission. In these two patients, the bleeding diathesis improved and the coagulation parameters normalized, confirming a causal relationship between the plasma cell dyscrasia and the AVWS. AVWS should be considered in AL amyloidosis patients with hemorrhagic diatheses and normal clotting factor levels.     

Tuberculous pleural effusion

Tuberculous effusion is a common disease entity with a spectrum of presentations from a largely benign effusion, which resolves completely, to a complicated effusion with loculations, pleural thickening and even frank empyema, all of which may have a lasting effect on lung function. The pathogenesis is a combination of true pleural infection and an effusive hypersensitivity reaction, compartmentalized within the pleural space. Diagnostic thoracentesis with thorough pleural fluid analysis including biomarkers such as adenosine deaminase and gamma interferon achieves high accuracy in the correct clinical context. Definitive diagnosis may require invasive procedures to demonstrate histological evidence of caseating granulomas or microbiological evidence of the organism on smear or culture. Drug resistance is an emerging problem that requires vigilance and extra effort to acquire a complete drug sensitivity profile for each tuberculous effusion treated. Nucleic acid amplification tests such as Xpert MTB/RIF can be invaluable in this instance; however, the yield is low in pleural fluid. Treatment consists of standard anti‐tuberculous therapy or a guideline‐based individualized regimen in the case of drug resistance. There is low‐quality evidence that suggests possible benefit from corticosteroids; however, they are not currently recommended due to concomitant increased risk of adverse effects. Small studies report some short‐ and long‐term benefit from interventions such as therapeutic thoracentesis, intrapleural fibrinolytics and surgery but many questions remain to be answered.     

Treatment of diuretic refractory pleural effusions with bevacizumab in four patients with primary systemic amyloidosis

Refractory pleural effusions present a challenging management problem and are associated with a poor prognosis in patients with primary systemic amyloidosis (AL). We report a series of four patients with AL who presented with bilateral pleural effusions that were refractory to diuretic therapy. After treatment with bevacizumab, an antivascular endothelial growth factor (VEGF) antibody, three of the four patients had improvement in their pleural effusions, peripheral edema, and functional status. Additional studies are needed to further define the role of bevacizumab in the management of this group of patients.     

Pulmonary anisakiasis presenting as eosinophilic pleural effusion

A 63-year-old man developed a pleural effusion with marked eosinophilia, which was more prominent in the pleural fluid than in the peripheral blood. The pleural effusion spontaneously disappeared 7 days after admission. A multiple dot enzyme-linked immunosorbent assay for anisakiasis was strongly positive for both the serum and pleural fluid. The serum IgG titre for Anisakis simplex gradually decreased over 7 months. It is suspected that Anisakis larvae can penetrate the alimentary canal, and then migrate into the pleural cavity through the diaphragm. Screening with a serological test is useful in the diagnosis of this condition; human pulmonary anisakiasis.     

非小细胞肺癌性恶性胸腔积液研究进展

恶性胸腔积液（malignantpleuraleffusion,MPE）约占临床所有胸腔积液的40％,多数MPE患者症状严重,预后差,总体生存期3～6个月,且现有治疗效果不佳。MPE的病因复杂,主要为肿瘤对胸膜的直接侵袭和转移。肺癌是MPE最常见的病因,约15％晚期非小细胞肺癌会发生MPE。本文将综述非小细胞肺癌所致MPE的发生机制,并详细介绍MPE诊疗和预后进展。     

结核感染T细胞斑点试验联合胸腔积液腺苷脱氢酶检测对结核性胸腔积液的诊断价值研究

目的?分析结核感染T细胞斑点试验（tuberculosis infection T cell spot test, T-SPOT.TB）结合胸腔积液生化检测对结核性胸腔积液的诊断价值。方法?对2019年2月—2022年2月期间就诊于河北省胸科医院的126例有肺部病灶伴胸腔积液患者展开研究,所有患者均完成T-SPOT.TB试验和入院当天的胸腔积液生化检测,依据是否存在结核杆菌感染将其分为结核组（n=48,确诊为结核性胸腔积液）和对照组（n=78,确诊为非结核性肺部病灶伴胸腔积液）。统计并比较2组患者的各项一般资料和临床资料,Logistic多因素分析结核性胸腔积液的危险因素,并应用ROC曲线分析胸腔积液T-SPOT.TB和胸腔积液腺苷脱氨酶（adenosine deaminase, ADA）及2者联合对结核性胸腔积液的诊断价值。结果?Logistic多因素分析结果显示,结核病接触史、结核性胸腔积液结核菌素试验阴性率较高、胸腔积液T-SPOT.TB阳性、胸腔积液ADA≥45 U/L为发生结核性胸腔积液的危险因素（P均＜0.05）。ROC曲线分析显示胸腔积液T-SPOT.TB和胸腔积液ADA诊断结核性胸腔积液的最佳临界值分别为276.43×106/ml和45.36 U/L,AUC分别为0.67和0.63,灵敏度分别为74.26％和69.26％,特异度分别为72.17％和68.84％,2者联合诊断的AUC为0.86,灵敏度为81.65％,特异度为79.43％。结论?T-SPOT.TB结合胸腔积液检测对诊断结核性胸腔积液患者有较佳价值。     

Rheumatoid pleural effusion

OBJECTIVES: To describe the clinical and laboratory features of rheumatoid pleural effusion (RPE) and the diagnostic and therapeutic approaches to this condition. METHODS: The review is based on a MEDLINE (PubMed) search of the English literature from 1964 to 2005, using the keywords "rheumatoid arthritis" (RA), "pulmonary complication", "pleural effusion", and "empyema". RESULTS: Pleural effusion is common in middle-aged men with RA and positive rheumatoid factor (RF). It has features of an exudate and a high RF titer. Underlying lung pathology is common. Generally RPE is small and resolves spontaneously but symptomatic RPE may require thoracocentesis. Rarely, RPE has features of a sterile empyematous exudate with high lipids and lactate dehydrogenase, and very low glucose and pH levels. This type of effusion eventually leads to fibrothorax and lung restriction. Superimposed infective empyema often complicates RPE. Oral, parenteral, and intrapleural corticosteroids, pleurodesis and decortication, have been used for the treatment of sterile RPE. Infected empyema is treated with drainage and antibiotics. CONCLUSIONS: RPE may evolve into a sterile empyematous exudate with the development of fibrothorax. Symptomatic effusions or suspicion of other causes of exudate (infection, malignancy) require thoracocentesis. The "rheumatoid" nature of the pleural exudate in patients without arthritis mandates a pleural biopsy to exclude tuberculosis or malignancy. The optimal therapy of RPE has yet to be established. The role of cytokines in the course of RPE and the possible usefulness of cytokine blockade in the treatment of this RA complication require further evaluation.     

以胸腔积液为首诊的肺栓塞19例临床分析

目的分析以胸腔积液为首诊的肺栓塞患者的临床特点,增强对症状不典型肺栓塞的认识,降低误诊率。方法回顾性分析19例以胸腔积液为首要临床表现的肺栓塞患者的临床特点。结果 71例肺栓塞患者中19例出现胸腔积液,占26.76%;大量胸腔积液1例,中量2例,少量16例;双侧胸腔积液2例,单侧胸腔积液17例,其中右侧8例,左侧9例;3例行胸腔穿刺胸水常规化验均为渗出性胸水。结论胸腔积液是肺栓塞的非典型、非特异性临床表现,临床上要提高警惕,及时完善D-二聚体检测,CT肺动脉造影检查,以提高确诊率。     

恶性胸膜间皮瘤合并肺结核/结核性胸腔积液的临床特征分析

目的:分析恶性胸膜间皮瘤(MPM)合并肺结核/结核性胸腔积液(TPE)患者的临床特征,以加强对该类疾病的认识,减少误诊误治。方法:收集首都医科大学附属北京胸科医院2012年2月至2020年2月MPM并肺结核/TPE患者病例资料14例,回顾性分析其临床症状、体征、实验室检查,胸部影像特征等,以总结其特点。结果:剧烈且进行...     

肺癌引起的恶性胸腔积液的研究和治疗进展

肺癌引起的恶性胸腔积液(malignant pleural effusion,MPE)发病率高且临床治疗效果不佳.出现MPE的肺癌患者生活质量明显下降,病死率明显增高.近期科学研究对MPE的病理生理过程进行了更多的阐述——宿主胸膜受肿瘤侵犯后分泌大量血管活性物质,促成了适合肿瘤生长的微环境和MPE的形成,同时肿瘤细胞通过激活某些信号通路、分泌重要的炎症介质来募集宿主细胞.同样地,宿主细胞也可激活细胞内信号通路、分泌炎症介质影响肿瘤细胞的功能.两者共同影响肿瘤的生长和MPE的产生.一些生物实验为MPE的形成提供了新的靶向治疗的方法,并且其得到的阳性结果为后续MPE的治疗提供了新的思路.     

肺癌患者胸水CA125、CEA测定及其临床意义

目的　探讨 CA1 2 5、CEA在良、恶性胸腔积液鉴别诊断中的价值。方法　对 36例恶性胸腔积液、34例良性胸腔积液于治疗前同时抽取血清及第一次胸腔积液标本进行 CA1 2 5、CEA测定。结果　恶性胸腔积液组血清和胸腔积液 CA1 2 5、CEA测定值高于良性胸腔积液组 (P<0 .0 1)。 CA1 2 5敏感度 5 8.3% (2 3/36 ) ,特异度 88.2 % (30 /34) ;CEA敏感度 6 8.3% (2 3/36 ) ,特异度 85 .3% (2 9/34)。CA1 2 5和 CEA联合检测敏感度 72 .2 % ,特异度 94 .1%。结论　胸腔积液 CA1 2 5和 CEA联合检测对恶性胸腔积液的诊断价值更大。     

Unexpandable lung from pleural disease

Unexpandable lung is a common complication of malignant pleural effusions and inflammatory pleural diseases, such as pleural infection (e.g. empyema and complicated parapneumonic effusion) and noninfectious fibrinous pleuritis. Unexpandable lung due to pleural disease may be because of an active pleural process, and is referred to as malignant or inflammatory lung entrapment. An unexpandable lung may also be encountered in the setting of remote pleural inflammation resulting in a mature fibrous membrane overlying the visceral pleura preventing full expansion of the lung. This condition is termed trapped lung and may be understood as a form of defective healing of the pleural space. Trapped lung typically presents as a chronic, stable pleural effusion without evidence of active pleural disease. An unexpandable lung most often manifests itself as an inability of fully expanding the lung with pleural space drainage. Patients will either develop chest pain preventing complete drainage of the pleural space or develop a post‐procedure pneumothorax. Pleural manometry and radiological imaging are useful in the assessment of an unexpandable lung. Pleural manometry can demonstrate abnormal lung expansion during drainage and imaging will demonstrate abnormal visceral pleural thickening found in trapped lung or malignant and inflammatory lung entrapment.