原发性肺淋巴瘤18例临床和影像及病理特点

目的 探讨原发性肺淋巴瘤(PPL)的临床表现、病理和影像学特点、诊断及治疗方法,提高其诊断率.方法 回顾性分析1989年1月至2007年2月北京协和医院住院的原发性肺淋巴瘤18例,总结其临床表现、病理和影像学特点、诊断手段、治疗方法及预后.生存时间按月记录,以手术日至患者最后一次就诊时间为准.计量资料采用秩和检验,计数资料采用卡方检验.使用SPSS 10.0统计软件进行数据分析.结果 18例中男6例,女12例,年龄17～71岁,中位年龄47.5岁.外科肺活检确诊15例(其中1例曾行经支气管镜活检发现肿瘤细胞,但未能明确病理类型),经皮肺活检确诊1例(1/6),经皮肺活检及经支气管镜活检标本共同确诊1例,经支气管镜活检确诊l例(1/10).病理结果为霍奇金淋巴瘤2例,其中混合细胞型和结节硬化型各1例;黏膜相关淋巴组织淋巴瘤9例,滤泡性淋巴瘤1例,弥漫性大B细胞淋巴瘤2例,间变性大细胞淋巴瘤2例,2例外院切片我院会诊后诊断为非霍奇金淋巴瘤.主要症状为咳嗽(11/18)和发热(6/18).18例中影像学表现为肺内结节或团块影14例,片状浸润影11例,实变影5例.胸腔积液和肺不张各5例,肺门或纵隔淋巴结肿大7例,空洞病变1例,病灶内液化1例.11例曾被误诊.3例手术完全切除病灶,8例单纯化疗,6例在肺叶切除术后化疗,1例放疗及化疗.中位随访时间为11个月(10 d至205个月),目前存活13例,失访4例,死亡1例.非霍奇金淋巴瘤患者白细胞异常升高与疾病进展(预后不良)相关,预后不良组中位数为25.1×109/L(18.1～39.1)×109/L,预后良好组中位数为6.7×109/L(5.48～8.41)×109/L,u=0.000,P＜0.05.结论 PPL临床表现不典型,易误诊;及时行外科肺活检获取组织病理标本有利于早期诊断.     

原发性肺黏膜相关淋巴组织淋巴瘤 1 例并文献复习简

原发性肺黏膜相关淋巴组织淋巴瘤（primary pulmonary mucosa-associated lymphoid tissue lymphoma,MALT）是一种少见的淋巴结外淋巴瘤,其缺乏特异的临床症状,常易误诊.现就我院1例经气管镜下活检病理确诊的原发性肺黏膜相关淋巴组织淋巴瘤患者的临床资料进行分析,并结合相关文献讨论,以提高对该病的诊治水平.     

原发性肺黏膜相关淋巴组织淋巴瘤1例并文献复习

<正>原发性肺黏膜相关淋巴组织淋巴瘤(primary pulmonary mucosa-associated lymphoid tissue lymphoma,MALT)是一种少见的淋巴结外淋巴瘤,其缺乏特异的临床症状,常易误诊。现就我院1例经气管镜下活检病理确诊的原发性肺黏膜相关淋巴组织淋巴瘤患者的临床资料进行分析,并结合相关文献讨论,以提高对该病的诊治水平。临床资料患者男性,59岁,因"间断咳嗽十月余。"于2012年09月19日入院。近10月来出现间断咳嗽,以吸入冷空气后咳嗽明显,干咳无痰,无胸痛、胸闷,     

伴胸部受累的淋巴瘤25例临床分析

目的 分析伴胸部受累的淋巴瘤的临床特点与诊断方法,以提高其诊断率.方法 回顾性分析2000-2007年北京大学第三医院确诊的有胸肺病变的淋巴瘤患者25例,收集患者确诊前的症状、体征、实验室检查结果、影像学资料、确诊方法和病理诊断.结果 25例患者中位年龄46岁,发热(13例)、6个月内体重减轻10%以上(11例)、咳嗽(10例)、气短(9例)、浅表淋巴结无痛性肿大(16例)是最常见的临床表现.72.7%的患者ESR增快;81%的患者血清乳酸脱氢酶(LDH)升高.25例患者中纵隔肺门淋巴结肿大者16例;影像学显示肺受累15例,包括斑片或实变表现、团块、多发结节、弥漫磨玻璃影、粟粒样病变;另外胸腔积液10例,心包积液4例,胸壁肿物2例;上述表现中有2种以上同时存在的患者18例.胸膜受累患者的胸腔积液外观呈黄色浑浊、血性或乳糜性,黏蛋白试验均阳性,比重1.031,白细胞6.72×10~9/L,淋巴细胞86%,中性粒细胞14%,蛋白31.4g/L,LDH 296 U/L,腺苷脱氨酶(ADA)67.4 U/L.外科手术活检确诊16例,其中浅表淋巴结活检确诊12例;超声或CT引导穿刺活检确诊5例;骨髓穿刺确诊1例;胸腔镜胸膜活检和纵隔镜纵隔肿物活检确诊各1例;经支气管镜黏膜活检确诊1例,而接受支气管镜气道黏膜活检和(或)经支气管肺活检(TBLB)确诊共8例.病理诊断除1例霍奇金淋巴瘤外,其余24例均为非霍奇金淋巴瘤.结论 伴胸部受累的淋巴瘤患者临床表现缺乏特异性,同时存在浅表或纵隔淋巴结肿大较为多见,血清学检查、胸腔积液性质和影像学检查亦有一定特点.浅表淋巴结手术活检是确诊淋巴瘤简便易行的方法;微创活检方法如超声或CT引导下浅表肿物、胸膜、肺、肝、脾、深部淋巴结活检及胸腔镜和纵隔镜胸膜、肺、纵隔病变活检亦具有较好的诊断价值,而经支气管镜获取气道黏膜和肺组织确诊率低.     

肺黏膜相关淋巴组织淋巴瘤MSCT表现与病理学基础

目的探讨肺黏膜相关淋巴组织(MALT)淋巴瘤的多层螺旋CT(MSCT)表现特征与病理学表现的关系,提高本病的影像诊断准确性。 方法收集我院17例经病理证实的肺MALT淋巴瘤患者的MSCT及病理资料,15例患者行肺部CT平扫及增强扫描,2例患者仅行肺部CT平扫,结合病理学基础分析其影像学表现特征。 结果17例肺MALT淋巴瘤中,多发5例(5/17),单发12例(12/17),病灶主要分布在支气管血管旁或胸膜下。影像表现为实变形6例(6/17,35%),肿块或结节形7例(7/17,41%),支气管血管束型2例(2/17,12%)混合型2例(2/17,12%)。其中,15例见充气支气管征,10例见血管造影征,9例伴胸腔积液,5例伴支气管扩张,4例伴纵膈淋巴结肿大,2例可见钙化灶,伴肺脓肿、空洞各1例。 结论肺MALT淋巴瘤CT表现以肺实变影、肿块影伴充气支气管征及血管造影征为主要特征,对本病的诊断及鉴别诊断具有重要的提示作用,但确诊需依靠病理活检。     

原发性肺黏膜相关淋巴组织淋巴瘤3例并文献复习

<正>黏膜相关性淋巴组织淋巴瘤(mucosa-associated lymphoid tissue lymphoma,MALToma)属淋巴结以外与黏膜组织相关的一种淋巴组织肿瘤性疾病。MALToma多发生于胃肠道,原发于肺少见[1-2],由于缺乏特异的临床症状及影像学表现,常易误诊。现将我科2010年至2013年诊治的3例原发性肺MALToma患者的临床资料进行分析,并结合相关     

北京协和医院对18年间病理诊断肺淋巴瘤患者的回顾分析

目的回顾性总结分析肺淋巴瘤的分类、分型特征及临床特点。方法收集北京协和医院1999年-2016年近18年期间病理诊断为肺淋巴瘤的全部病例,通过光镜、免疫组织化学染色及基因重排方法进行病理分析。结果本组共有肺淋巴瘤病例142例,男女比例1︰1.33,平均年龄48岁。123例(86.6%)诊断为非霍奇金淋巴瘤,19例(13.4%)诊断为霍奇金淋巴瘤。101例(69.2%)诊断为B细胞性淋巴瘤,其中45.6%(46例)为黏膜相关淋巴组织淋巴瘤或边缘区淋巴瘤,18.8%(19例)为弥漫大B细胞性淋巴瘤,11.9%(12例)为淋巴瘤样肉芽肿病。22例诊断为T细胞性淋巴瘤,其中27.3%为外周T细胞淋巴瘤(6例),13.6%(3例)为NK/T细胞淋巴瘤,9.1%(2例)为间变大细胞淋巴瘤。121例患者临床资料完善,其中64例(52.9%)为原发性肺淋巴瘤,男女比例1︰1.37,平均年龄50岁;57例(47.1%)为继发性肺淋巴瘤,男女比例1︰1.19,平均年龄45岁。在原发性肺淋巴瘤中,96.9%为非霍奇金淋巴瘤,3.1%为霍奇金淋巴瘤;在继发性肺淋巴瘤中,78.9%为非霍奇金淋巴瘤,21.1%为霍奇金淋巴瘤。65例手术切除或胸腔镜下肺活检病例全部进行了病理分类及分型,77例穿刺活检病例中有22例(28.6%)进一步分型困难。结论肺淋巴瘤中最常见类型依次为黏膜相关淋巴组织淋巴瘤、霍奇金淋巴瘤、弥漫大B细胞性淋巴瘤和淋巴瘤样肉芽肿病,其中黏膜相关淋巴组织淋巴瘤和淋巴瘤样肉芽肿病是主要的原发性肺淋巴瘤类型,而弥漫大B细胞性淋巴瘤和霍奇金淋巴瘤是主要的继发性肺淋巴瘤类型。开胸手术或胸腔镜下肺活检的病例易于明确病理分型,而穿刺活检病例中有小部分病例进一步分型困难。     

肺黏膜相关淋巴组织淋巴瘤合并非结核分枝杆菌肺病1例并文献复习

<正>黏膜相关淋巴组织(mucosa-associated lymphoid tissue, MALT)淋巴瘤是非霍奇金淋巴瘤的一个独特亚型，约占所有非霍奇金淋巴瘤的5%。肺MALT淋巴瘤罕见，占原发性肺恶性肿瘤0.5%^[1]。肺MALT淋巴瘤起源于支气管黏膜相关淋巴组织(Bronchus-Associated Lymphoid Tissue, BALT)是肺原发淋巴瘤的主要组织类型^[2]。     

肺原发性黏膜相关淋巴组织淋巴瘤12例诊断与治疗

目的探讨肺原发性黏膜相关淋巴组织淋巴瘤的临床诊断与治疗方法。方法对1992年8月至2005年5月收集的12例肺原发性黏膜相关淋巴组织淋巴瘤（MALT lymphoma）患者的临床资料、治疗、随访结果进行总结。结果随访6～164个月（平均70．3个月），12例目前均存活。手术治疗6例，术后化疗4例。单纯化疗4例，2例为完全缓解，2例为部分缓解。临床观察2例，带病生存。1例术后12．7年复发，随访8个月生存良好。结论肺原发性黏膜相关淋巴组织淋巴瘤临床表现无特异性，但其胸部放射线检查较为特异，表现为边缘模糊，内有充气支气管征的肿块。该病主要依靠病理组织学及免疫组化诊断。手术治疗结合化疗或者单纯化疗，疗效均较满意，是一种预后相对良好的恶性肿瘤。     

原发性肺黏膜相关淋巴组织淋巴瘤4例临床病例分析

目的 总结原发性肺黏膜相关淋巴组织(MALT)淋巴瘤的临床表现、影像学特点、诊断手段、治疗方法及预后,提高临床诊治水平.方法 回顾性分析4例经病理确诊的原发性肺MALT淋巴瘤的临床资料并随诊分析预后.结果 4例原发性肺MALT淋巴瘤患者均为老年女性,为原发性肺非霍奇金淋巴瘤的最常见类型.其中3例为查体发现,1例因咳嗽、咳痰和痰中带血就诊.发病时间为7个月至5年.肺部体征无特异性.血炎症指标和肿瘤相关指标多为正常.影像学以肿块影和结节为主要表现.患者肺通气和弥散功能正常.经胸腔镜、CT引导下肺穿刺、开胸手术获取病变组织而确诊.病理表现为弥漫浸润生长的小淋巴细胞,可见淋巴上皮增生.治疗主要是化疗和手术.随访1～8.8年,1例随访4.5年时可疑复发,其余3例均病情稳定.结论 肺原发性MALT淋巴瘤为少见病,好发于老年女性,起病隐匿,临床表现不典型.诊断须靠有经验的病理专家作出.治疗尚无指南,无症状者可采取“观察等待”策略,待肿瘤进展或出现症状时,首选苯丁酸氮芥化疗,联合利妥昔单抗与否均可.预后良好,但复发率高.     

Bilateral pulmonary nodules as a manifestation of primary pulmonary T-cell lymphoma

Primary pulmonary lymphoma (PPL) is an uncommon pathology and is usually of the B-cell type, originating in lymphoid tissue associated with the bronchial mucosa (MALT/BALT lymphoma). Very few cases of T-cell PPL, the majority diagnosed by open lung biopsy, have been described in medical literature. We report a case of an immunocompetent patient with fever and bilateral pulmonary nodules, diagnosed with T-cell PPL by transbronchial biopsy. The patient’s condition deteriorated and she responded poorly to chemotherapy. PPL should be included in the differential diagnosis of patients with fever and bilateral pulmonary nodules.     

Gastric mucosa‐associated lymphoid tissue lymphoma and Helicobacter pylori infection

OBJECTIVE: Numerous studies have demonstrated the role of Helicobacter pylori infection in the pathogenesis of gastric MALT lymphoma and the present study aimed to analyze this correlation in Chinese patients. METHODS: Thirty‐five cases of primary gastrointestinal non‐Hodgkin's lymphoma that had been surgically resected and pathologically examined during the past 20 years were collected. The tissue samples were re‐examined by a physician from the pathology department. Immunohistochemical staining and H. pylori tests were conducted. The clinical diagnosis of gastric MALT lymphoma and the results of therapy were analyzed. RESULTS: According to the immunohistochemistry results, 21 cases were MALT lymphoma, and of these 16 were gastric MALT lymphoma, one was intestinal MALT lymphoma and four were colonic MALT lymphoma. Of the 16 samples of gastric MALT lymphoma, 13 were positive for H. pylori infection and three could not be evaluated because the sample was full of cancer cells. Of the cases of gastric MALT lymphoma, two were stage I₁, five were stage II₁, and nine were stage II_E. Eleven patients underwent endoscopy: three were misinterpreted as gastric carcinoma, one was diagnosed as Menetrier's disease, one as chronic atrophic gastritis, and only six cases were correctly diagnosed before surgery. Eradication of H. pylori in one patient with gastric MALT lymphoma resulted in regression of the lesion; that patient was followed up for 3 years without relapse. Fifteen cases underwent surgery and 10 were followed up for 5 years: four relapsed within 1?2 years after operation and six remained well. CONCLUSION: There is a close relationship between H. pylori infection and gastric MALT lymphoma. Early gastric MALT lymphoma can be cured after eradication of H. pylori. The depth of the lesion should be diagnosed by echoendoscopy and therapy should be chosen on the basis of the stage of the disease.     

Lymphomes spléniques de la zone marginale et auto-immunité : à propos de six cas

Introduction

Autoimmune manifestations are common in splenic marginal zone lymphoma (SMZL) and are sometimes the presenting feature of the disease. Autoimmune cytopenia (anemia, thrombocytopenia) are the most frequently reported autoimmune conditions. However, other immunological manifestations may be associated with SMZL.

Méthodes

We report a retrospective case series of six patients with SMZL associated with autoimmunity.

Results

Auto-immune manifestations were the presenting feature of lymphoma in four cases. Auto-immune manifestations included auto-immune cytopenia in three cases (two hemolytic anemia and one pancytopenia), thyroiditis in two cases, systemic lupus and Still's disease in one case each. Antinuclear antibodies were detected with a titre of 1/250 in three cases, and with a titre of 1/32,000 in the patient with systemic lupus. Testing for DNA antibodies was negative in all cases. Two patients had a circulating lupus anticoagulant, with portal venous thrombosis following splenectomy in one case. One patient had hypogammaglobulinemia. A monoclonal gammopathy was detected in three patients. All patients had spleen enlargement. Immunophenotyping of blood peripheral lymphocyte was typical in five out of the six cases. Bone marrow was infiltrated in five out of the six cases. Diagnosis was obtained by the combination of immunophenotyping and bone marrow histopathology in five cases, and by splenic histopathology in the remaining case. Hepatitis C virus serology was negative in all patient.

Conclusion

Autoimmune disease as systemic lupus or Still's disease may be associated with SMZL before its tumoral manifestations are evident. In this mode of presentation, spleen enlargement, hypogammaglobulinemia, monoclonal gammopathy, and multiple autoimmune diseases, should alert the physician.     

Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis.

This study investigated interstitial pneumonia associated with amyopathic dermatomyositis, dermatomyositis and polymyositis, paying particular attention to muscular and/or cutaneous manifestations and their chronology relative to lung involvement. Patients included four males and 13 females, aged 51.7+/-10.8 yrs, who had surgical lung biopsy. Diagnoses included dermatomyositis (10 patients), polymyositis (four patients) and amyopathic dermatomyositis (three patients). Solitary respiratory manifestations preceded the onset of any skin or muscle disease in four cases (24%). Reticular and ground glass opacities were the most frequent computed tomography (CT) findings. Pathological review showed nonspecific interstitial pneumonia (eleven, 65%; cellular, two; cellular and fibrotic, five; fibrotic, four), usual interstitial pneumonia (two), organising pneumonia (two), lymphocytic interstitial pneumonia (one), and unclassifiable interstitial pneumonia (one). Nonspecific interstitial pneumonia was the most common histological pattern of interstitial pneumonia in patients with amyopathic dermatomyositis (three of three) and in patients with respiratory symptoms as the initial clinical manifestation of the connective tissue disease (three of four). Survival at 5 yrs was 50%. This study shows the clinician should remain alert to potential muscular or cutaneous manifestations whenever a pathological diagnosis of nonspecific interstitial pneumonia is made.     

109例肺泡蛋白沉积症临床影像及病理特征分析

目的肺泡蛋白沉积症为肺部少见疾病,通过本组病例的学习,提高大家对肺泡蛋白沉积症的认识,减少误诊和漏诊。方法收集2011年01月至2019年03月在南京大学医学院附属鼓楼医院呼吸与危重症医学科经病理学明确诊断为肺泡蛋白沉积症的109例患者临床资料,分析其临床特点、胸部HRCT及病理学表现。结果本组患者平均年龄48±11岁(20~73岁),其中男68例,女41例。临床表现:以活动后气喘(70例)和咳嗽伴或不伴咳痰(64例)最多见,胸闷27例,胸痛7例,发热6例,咯血1例。另外体检发现7例。实验室检查:血CEA升高8例,NSE升高49例,CYFRA21-1升高67例;合并有自身抗体异常14例;血甘油三脂增高25例;血气分析示低氧血症患者共54例。HRCT主要表现:典型肺泡蛋白沉积症表现(铺路石症和地图样分布)共102例,实变影6例,细网状阴影6例,纵隔淋巴结肿大2例,结节影2例,胸腔积液1例,胸膜增厚1例。所有患者均进行了病理学检查,肺泡灌洗液PAS染色阳性,和或肺活检组织示肺泡腔内无定形嗜伊红物沉积且PAS染色阳性。其中经支气管镜肺活检(TBLB+BAL)85例,CT引导下经皮肺穿刺3例,外科胸腔镜2例,支气管镜冷冻肺活检(TBLC)2例;另外17例经肺泡灌洗检查明确诊断。结论肺泡蛋白沉积症患者的临床表现多样,缺乏特异性,绝大多数患者HRCT表现典型,确诊仍需病理。     

Pulmonary zygomycosis: a radiographic and clinical spectrum.

Eight documented cases of pulmonary zygomycosis were analyzed retrospectively with regard to radiographic and clinical features. Predisposing factors were diabetes mellitus in six cases, lymphoblastic lymphoma in one case, and surgery to correct a tracheoesophageal fistula in one case. Two of the patients with diabetes had also undergone renal transplantation for diabetic nephropathy and were immunosuppressed. The more usual radiographic findings of pulmonary zygomycosis represent a spectrum that comprises a normal chest radiograph, a lung abscess, subacute or chronic pneumonia that often evolves into a lung abscess, and rapidly progressive fatal pneumonia. Awareness of the various presentations of pulmonary zygomycosis is important because early diagnosis and appropriate therapy clearly have been shown to improve the survival rate of these patients. Zygomycosis should be included in the differential diagnosis when patients with diabetes mellitus, patients with leukemia or lymphoma, or immunocompromised patients present with or develop perplexing pulmonary abnormalities.     

Cavitary pulmonary involvement of diffuse large B-cell lymphoma transformed from extra nodal marginal zone B-cell lymphoma MALT type

We describe a case of pulmonary diffuse large B-cell lymphoma (DLBCL), which was thought to arise from extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). A 68-year-old woman presented with a 2-month history of cough and bloody sputum. The chest X-ray and computed tomography revealed a mass with cavitation in the right lower lobe. Transbronchial biopsy specimens revealed a granulomatous infiltration without malignant cells. However, diagnosis of MALT lymphoma was established from gastric biopsy specimen. Subsequently, a right lower lobectomy was performed because of hemoptysis. Examination of the resected specimen revealed a diffuse large B-cell lymphoma, which was considered to have transformed from MALT lymphoma, because both lung and stomach lesions had the chromosomal translocation t(11;18)(q21;q21) in common. In addition, there were no nodules, masses, alveolar or interstitial infiltrates in the lung fields, which are usually observed in the case of marginal zone B-cell lymphoma of bronchial mucosa-associated lymphoid tissue. These findings indicate that involvement of DLBCL have to be considered in patients with MALT lymphoma and cavitary lesion of the lung.     

肺黏膜相关淋巴组织淋巴瘤的多层螺旋CT表现

目的分析肺黏膜相关淋巴组织(mucosa-associated lymphoid tissue, MALT)淋巴瘤多层螺旋电脑断层扫瞄(multilayer spiral computed tomography, MSCT)影像表现及特征，提高对该病的认识及诊断准确性。 方法回顾性分析16例经病理证实肺MALT淋巴瘤患者的MSCT表现。 结果16例肺MALT淋巴瘤患者中单发6例，多发10例。根据MSCT病变形态分布，将16例MALT病变分为三类：结节/肿块型(7例)，渗出/实变型(6例)，混合型(3例)。病变周围血管支气管束增粗14例，8例病灶可见钙化。空气支气管征11例，其中9例支气管扩张。4例结节/肿块型呈"钻缝"样生长。16例中有13例行增强扫描，其中11例均匀强化，11例可见血管造影征。3例纵隔淋巴结增大，8例合并胸水。 结论肺MALT淋巴瘤MSCT影像学表现具有一定特征性，结合临床惰性生长表现，可提示肺MALT淋巴瘤的诊断。