组织细胞坏死性淋巴结炎

组织细胞坏死性淋巴结炎是一种具有特殊临床病理过程的自限性淋巴结疾病,以顽固性发热、区域性淋巴结肿大和一过性白细胞减少为特征。病因及发病机制不明,临床表现具有非特异性,组织学形态较复杂,容易误诊。抗生素治疗对本病无效,而糖皮质激素是最有效的治疗药物,大多预后良好。     

组织细胞坏死性淋巴结炎一例报告并文献复习

目的探讨组织细胞坏死性淋巴结炎（HNL）的临床特征,分析相关实验室检查结果。方法我院呼吸科2008年收治1例HNL患者,结合文献报道477例共478例进行回顾分析。结果478例HNL可见于各年龄段,男女比例为0．88：1。临床表现颈部淋巴结肿大占96．4％（325／337）,发热占93．8％（436／465）,可伴有肝肿大20．7％（61／294）,脾肿大22．0％（68／309）和皮疹20．5％（87／425）。实验室检查：72．1％（305／423）一过性白细胞计数下降,27．6％（74／268）贫血,87．5％（309／353）血沉增快。7．6％（12／158）抗核抗体阳性,4．6％（3／65）抗DsDNA抗体阳性。17．0％（15／88）EB病毒IgM抗体阳性,50．0％（51／102）EB病毒IgG抗体阳性,4．5％（2／44）支原体抗体阳性,12．5％（5／40）巨细胞病毒IgM抗体阳性,18．8％（3／16）巨细胞病毒IgC．抗体阳性,10．6％（13／123）柯萨奇病毒IgM抗体阳性。7．8％（19／244）复发。结论HNL可能为多种病原体感染导致的自限性自身免疫性疾病,以长期发热、颈部淋巴结肿大为主要特征,可引起多脏器损害,可复发,确诊需淋巴结活检病理检查,抗生素治疗无效,激素及非甾体抗炎药治疗有效。     

组织细胞坏死性淋巴结炎

目的提高对少见病组织细胞坏死性淋巴结炎的认识。方法对我科诊治的患者进行分析,并结合文献复习。结果组织细胞坏死性淋巴结炎是一种具有特殊临床病理过程的自限性淋巴结疾病,病因及发病机制不明,以顽固性发热、区域性淋巴结肿大和一过性白细胞减少为特征。结论临床表现具有非特异性,组织学形态较复杂,容易误诊。抗生素治疗对本病无效,而糖皮质激素是最有效的治疗药物。     

组织细胞坏死性淋巴结炎52例临床病理表现

目的探讨组织细胞坏死性淋巴结炎的临床和病理学特点。方法回顾性分析52例组织细胞坏死性淋巴结炎的临床表现、淋巴结活检病理学特点及其诊治。结果52例患者中女性41例（79％）,主要表现为持续发热（100％）,单发（23％）或多发（77％）淋巴结肿大（以颈部多见）,多形性皮疹（35％）,外周血白细胞计数降低（76％）,血沉增快（100％）,抗生素治疗无效（100％）,小剂量肾上腺糖皮质激素治疗有效（81％）等。26例患者（50％）肝酶升高,仅7例（13％）伴流感样上呼吸道症状。淋巴结活检病理学特点为不同程度的凝固性坏死伴多种形态的组织细胞、淋巴细胞浸润,无中性粒细胞浸润。免疫组化染色示组织细胞CD68及T细胞CD3、CD45,RO阳性,CD15、CD20及CD30均阴性。结论组织细胞坏死性淋巴结炎的临床表现无特异性,较易误诊,确诊主要依靠病理活检及免疫组化检查。     

Expression of apoptosis-associated protein RCAS1 in macrophages of histiocytic necrotizing lymphadenitis

Receptor-binding cancer antigen expressed on SiSo cells (RCAS1), which is recognized by the 22-1-1 monoclonal antibody (MoAb) against human uterine adenocarcinoma cell line SiSo, has been identified on various kinds of cancer cells. RCAS1 appears to be an apoptosis-associated protein that induces apoptosis in activated T-cells and erythroid progenitor cells. We previously demonstrated that monocytes/macrophages express RCAS1. In the present study, we investigated RCAS1 expression by 22-1-1 MoAb in histiocytic necrotizing lymphadenitis (HNL), which is characterized by necrotic lesions consisting of T-cells undergoing apoptosis and macrophages in proliferation. Expression of RCAS1 was analyzed by immunohistochemical staining in 9 cases of HNL and in 9 cases of reactive lymphadenitis used as a control. The ratio of RCAS1+ cells to CD68+ cells (monocytes/macrophages) was significantly higher in the patients with HNL than in the patients with reactive lymphadenitis (P = .0002; paired t test). Our findings suggest that RCAS1 expressed on macrophages may play an important role in the induction of activated T-cell apoptosis in cases of HNL.     

可溶性Fas,可溶性Fas配体与细胞因子在1型糖尿病发病中的意义

目的　研究可溶性Fas(sFas)和可溶性Fas配体(sFasL)与细胞因子在1型糖尿病发病中的作用。方法　32名1型糖尿病患者和20名正常人的血清,采用夹心BAS-ELISA法分别检测sFas,sFasL,γ干扰素(IFN-γ)及白细胞介素-1(IL-1)含量。结果　1型糖尿病血清中sFas,IFN-γ及IL-1含量分别为(1　　546±685,1　　074±451与1　　406±721)ng/L,显著高于正常组;sFasL为(211±73)mg/L,低于正常组但差异无显著性。在1型糖尿病患者中高浓度sFas伴高IFN-γ者共12例,低浓度sFas伴低IFN-γ者共9例。结论　1型糖尿病患者血清中的sFas,IFN-γ及IL-1高于正常人,sFas与IFN-γ浓度呈正相关(r=0.79,P＜0.05)。对1型糖尿病患者血清进行sFas,IFN-γ及IL-1等检测可作为反映胰岛细胞病变的辅助指标,有助于对疾病的诊断与治疗。     

血清sFas、sFasL水平与特发性血小板减少性紫癜的关系

采用酶联免疫夹心法(ELISA)检测了25例特发性血小板减少性紫癜(ITP)患者血清中sFas、sFasL的水平.结果显示ITP患者血清sFas含量为16.51±6.86μg/L,sFasL含量为0.48±0.33μg/L,均显著高于正常对照(分别为P<0.001和P<0.01);18例ITP患者血清sFas水平升高,其中有9例同时有sFasL水平升高,但是血清sFas水平升高组的血小板计数与sFas正常组无显著差异.提示sFas和sFasL的异常参与了ITP的免疫病理过程.     

组织细胞坏死性淋巴结炎13例临床和病理分析

目的 通过对13例组织细胞坏死性淋巴结炎(HNL)患者的临床和病例资料进行总结分析,以探讨其临床和病理特点.方法 回顾性分析1997年6月～2006年12月住院治疗的13例HNL患者的临床及病理资料.结果 所有患者均有发热和颈部淋巴结大,大多数患者白细胞减少、淋巴细胞比率增高、血沉增快,9例EBV-DNA阳性.初诊时7例误诊为淋巴瘤,4例误诊为淋巴结核,1例误诊为败血症.淋巴结活检HE染色可见典型的病理学改变.12例患者应用疗程为2～6个月的肾上腺皮质激素治疗,疗效显著,未复发.1例应用肾上腺皮质激素2周治愈,但6年后复发.结论 该病临床表现缺乏特异性,易误诊,确诊需进行淋巴结活检,CD标志免疫组化为关键依据,可用于排除T、B淋巴瘤,其病因可能与EB病毒感染有关,早期长疗程的激素治疗可能有助于减少复发.     

Elevated serum interferon γ and interleukin-6 in patients with necrotizing lymphadenitis (Kikuchi's disease)

We investigated serum levels of interferon α, interferon γ, tumour necrosis factor α, interleukin-2 (IL-2) and interleukin-6 (IL-6) in patients with necrotizing lymphadenitis (Kikuchi's disease) (NL). Four male patients, diagnosed as having NL following biopsy of the affected lymph nodes and by the clinical course, were included in this study. All four patients had higher than normal serum interferon γ and IL-6 levels during the acute phase, which returned to normal levels during the convalescent phase. Interferon α, tumour necrosis factor α and IL-2 were, however, within the normal ranges. Our findings indicate the possibility that interferon γ and IL-6 may play an important role in the pathogenesis of NL.     

狼疮肾炎患者血清sFas和sFasL的变化

目的　研究狼疮肾炎患者血清sFas和sFasL的变化及意义。方法　采用双抗体夹心酶联免疫吸附法 (ELISA)检测正常对照 18例和狼疮肾炎患者 45例血清sFas和sFasL水平。结果　狼疮肾炎患者sFas和sFasL水平分别为 (14± 7) μg/L和 (0 0 7± 0 0 4) μg/L ,与正常对照组 (2 9±1 2 ) μg/L和 (0 0 5± 0 0 1) μg/L相比 ,差异有高度显著性意义 (P <0 0 1)。活动期sFas水平较缓解期明显增高 ,分别是 (17± 7) μg/L和 (9± 4) μg/L ,P <0 0 1;但sFasL活动期与缓解期差异无显著意义。狼疮肾炎血清sFas水平在白细胞减少、贫血、大量蛋白尿、肾功能减退、补体降低、ANA和抗RNP Ab阳性时升高 ;而sFasL仅在肾功能异常时减低 ,与其他指标没有明显相关性。结论　sFas及sFasL参与了狼疮肾炎的发生 ,sFas可作为狼疮肾炎的活动性实验室指标。     

组织细胞性坏死性淋巴结炎14例分析及文献复习

目的：探讨组织细胞性坏死性淋巴结炎的临床特点、组织病理学改变、诊断和治疗。方法：结合文献,回顾性分析1995－1999年收住我院内科的14例本病患者。结果：14例患者临床表现以发热、淋巴结肿大和粒细胞减少最常见,淋巴结组织病理学改变主要为副皮质区的多处坏死,不同程度的组织细胞、免疫母细胞、浆细胞样单核细胞增生,无中性粒细胞浸润,易引起误诊,尤其是误诊为淋巴结结核。结论：组织细胞性坏死性淋巴结炎具有典型的组织病理学改变,应注意防止误诊。     

组织细胞坏死性淋巴结炎29例临床分析及文献复习

目的 探讨组织细胞坏死性淋巴结炎的临床特点和诊治方法.方法 回顾近10年我院收治的29例组织细胞坏死性淋巴结炎的临床表现、实验室检查结果和治疗效果.结果 29例病人男女比例为14:15,100%有淋巴结肿大,72.4%有发热,44.8%患者有咽痛、咳嗽等,超声发现脾肿大者占46.7%(7/15),肝功能异常者34.6%(9/26),白细胞降低者占39.3%(11/28),血沉增快者占59.1%(13/19),C反应蛋白升高者占66.7%(10/15),ANA阳性率9.1%(1/11),抗-EBV IgM阳性率3.3%(3/9),16例患者接受了皮质激素治疗,10例患者自行缓解,另有1例出院后复发而服用皮质激素、1例半年后诊断为成人斯提尔病、1例不除外合并结缔组织病.结论 本病临床表现无特异性,诊断依赖淋巴结活检,治疗以糖皮质激素为首选,部分病人可自愈.     

Analysis of the clinical manifestations and 18F-FDG PET-CT findings in 40 patients with histiocytic necrotizing lymphadenitis

Histiocytic necrotizing lymphadenitis (HNL) is a rare, benign, and self-limiting inflammatory disease that mainly involves the lymph nodes. There is a lack of large sample studies concerning the clinical manifestations and imaging features of 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) of HNL.The clinical symptoms, laboratory examination results, 18F-FDG PET/CT imaging features, and treatment outcome were investigated in this retrospective study.A total of 40 HNL patients were recruited. The onset age was between 14 and 65 years, with a median of 25 years. The white blood cell count was 3.9 (2.9, 7.1) × 10⁹/L, C-reactive protein level was 20.2 (6.6, 63.8) mg/L, erythrocyte sedimentation rate was 29.0 (18.0,45.0) mm/h, and ferritin was 616.5 (205.6, 2118.1) ng/mL. An abnormal liver function was observed in 23 patients. 18F-FDG PET-CT showed that an abnormal lymph node metabolism was observed in 38 patients, among which the highest 18F-FDG maximal standard uptake value (SUVmax) of the lymph nodes ranged between 3.4 and 41.9; the nodes were mainly distributed in the neck and axilla regions. Meanwhile, a total of 2502 lymph nodes (721 lymph nodes with a short axis greater than 10 mm) were found in the 38 patients, including 1837 lymph nodes with an 18F-FDG SUVmax ≥ 2.5. The 18F-FDG SUVmax of the spleen ranged from 2.5 to 9.2 in 20 patients, while that of central and peripheral bone marrow ranged from 2.7 to 36.0 in 30 patients. After follow-up for an average period of 1 month, the symptoms improved after prednisone treatment.HNL often occurs in adolescents. Scanning with 18F-FDG PET/CT showed that most patients had multiple involved lymph nodes that were hypermetabolic, and only few lymph nodes are enlarged. Besides, the spleen or central and peripheral bone marrow could sometimes be hypermetabolic. Glucocorticoid treatment for the HNL patients is effective.     

慢性心力衰竭患者的血清可溶性Fas配体和可溶性Fas受体

目的 分析血清可溶性Fas配体(sFasL)和可溶性Fas受体(sEas)与慢性心力衰竭(CHF)的相关性。方法采用酶联免疫吸附双抗体夹心法检测33例CHF患者(CHF组,心功能Ⅱ-Ⅳ级,NYHA)血清sFasL和sFas浓度,并与18例心功能Ⅰ级(NYHA)组比较。结果 CHF与心功能Ⅰ级间sFasL浓度无显著统计学差异[231.50±84.50(心功能Ⅱ级216.50±96.00,Ⅲ级226.80±85.70,Ⅳ级244.00±73.00)vs217.50±89.00pg/mL,P>0.05]。而CHF组血清sFas浓度显著高于心功能Ⅰ级组[1353.30±507.71(心功能Ⅱ级1154.85±371.20,Ⅲ级1412.88±493.62,Ⅳ级1875.67±806.10)vs983.11±461.26pg/mL,P<0.05]。结论 血清sFasL与CHF无相关性。而血清sFas与CHF存在显著相关性。且sFas浓度增高的程度与CHF的严重程度相平行,sFas浓度增高可能在CHF发病机制中起重要作用。     

心力衰竭患者血浆凋亡相关因子水平的变化及其临床意义

目的 :探讨作为凋亡抑制因子Fas (sFas)和凋亡诱导因子Fas配体 (sFasL)在心力衰竭患者血浆中的水平及其临床意义。方法 :采用酶联免疫吸附法测定 6 7例心力衰竭患者 (心力衰竭组 )和 2 0例健康人 (正常对照组 )血浆中的sFas、sFasL、肿瘤坏死因子α、白细胞介素 6水平。结果 :心力衰竭不同心功能状态患者血浆sFas、肿瘤坏死因子α水平均高于正常对照组 ,心功能Ⅲ级、Ⅳ级患者中sFas水平 ,心功能Ⅲ级患者白细胞介素 6水平亦高于正常对照组 (P <0 0 5～ 0 0 1) ,并随心功能下降而上升 ,与心功能状态呈负相关 ,其水平与原发病无关。sFas和肿瘤坏死因子α水平在心功能Ⅱ级和Ⅳ级患者间比较有显著差异 (P <0 0 5 )。结论 :心力衰竭患者血浆中的sFas和sFasL的水平显著升高 ,提示sFas和sFasL水平升高乃至整个Fas/FasL系统可能在心力衰竭的发病中起着重要作用     

Apoptosis in the intestinal mucosa of patients with inflammatory bowel disease: evidence of altered expression of FasL and perforin cytotoxic pathways

Background and aims Abnormal apoptosis may result in the persistence of activated intestinal T-cells in inflammatory bowel disease (IBD). We investigated apoptosis in distinct mucosal compartments, and the expression of Fas/Fas ligand and perforin in the inflamed and non-inflamed intestinal mucosa of patients with IBD.Methods Colon specimens from 15 patients with ulcerative colitis (UC) and inflamed and non-inflamed mucosa from 15 patients with Crohns disease (CD) were analysed for densities and distribution of apoptotic cells determined by the terminal deoxynucleotidyltransferase-mediated dUDP-biotin nick-end labelling (TUNEL) method. Fas, FasL, and perforin-expressing cells were assessed by immunoperoxidase, and with anti-CD3, anti-CD20 and anti-CD68, by double immunofluorescence with confocal microscopy. Quantitative analysis was performed using a computer-assisted image analyser.Results Colonic lamina propria (LP) and epithelium from patients with UC showed higher rates of apoptosis than controls, but no difference was shown regarding patients with CD. In LP, co-expression of Fas was reduced with T-cells in inflamed CD mucosa, and with macrophages in all patients with IBD. No difference was found in the expression of Fas on B-cells. Rates of FasL-expressing cells in LP were higher in IBD than in controls, with no correlation with the rates of apoptosis. Rates of perforin-expressing cells in LP were greater in UC than in controls, and correlated to the rates of apoptosis. No difference was shown regarding the inflamed and non-inflamed CD mucosa. Rates of FasL and perforin-expressing intra-epithelial lymphocytes showed no difference among groups.Conclusions Increased expression of FasL in IBD colonic LP not parallelled by Fas on T-cells and macrophages may indicate a reduced susceptibility to the Fas/FasL-mediated apoptosis of lymphoid cells. Expression of perforin is correlated to the tissue damage, and may represent the enhancement of a distinct cytotoxic pathway in UC.Heitor S.P. Souza and Claudio J.A. Tortori contributed equally to this paper     

Distinct patterns of mucosal apoptosis in H pylori-associated gastric ulcer are associated with altered FasL and perforin cytotoxic pathways

INTRODUCTION Infection with H pylori induces chronic gastritis in virtually all infected hosts and it has been implicated as the major etiologic factor in peptic ulcer disease, chronic atrophic gastritis[1,2], gastric carcinoma, and gastric lymphoma[3,4].…     

Parvovirus B19-associated haemophagocytic syndrome with lymphadenopathy resembling histiocytic necrotizing lymphadenitis (Kikuchi's disease)

A 15-year-old girl developed a haemophagocytic syndrome caused by human parvovirus B19 (PVB19). The cervical lymph node histology, resembling that of histiocytic necrotizing lymphadenitis (HNL, Kikuchi's disease), included several transformed lymphocytes, numerous histiocytes, and massive necrosis. We detected PVB19-positive cells in the lymph node by immunohistochemistry. Possible autoimmune mechanisms in HNL-like diseases triggered by PVB19 are discussed.     

38例急性乙型病毒性肝炎肝组织中Fas抗原和Fas配体的表达研究

目的:探讨Fas-FasL系统在急性病毒性肝炎发病中的作用.方法:采用免疫组织化学技术.结果:38例急性乙型肝炎肝组织中的Fas和FasL表达的检出率分别为55.3%和66.5%;Fas和FasL的表达强度与患者的年龄、性别和血清A、A/G和PTA无关,而与血清ALT和TBIL水平有关.较重型(伴桥样坏死)的急性乙型肝炎其Fas和FasL表达比轻型急性乙型肝炎要强.结论:由CTL-Fas-FasL系统介导的肝细胞凋亡在急性乙型肝炎的发病中起了较重要的作用.     

sFas与sFasL在自身免疫疾病中的意义

目的　研究sFas与sFasL在系统性红斑狼疮 (SLE)等自身免疫疾病中的意义及抗单链DNA(ssDNA)抗体与sFas和sFasL介导凋亡的相关性。方法　采用夹心ELISA方法检测 31例SLE病人 ,32例类风湿关节炎 (RA)病人 ,2 0例 1型糖尿病 (IDDM )病人及 5例多发性硬化病 (MS)病人血清中sFas与sFasL含量及抗ssDNA抗体水平。结果　在SLE、RA、IDDM及MS患者血清中的sFas含量 (pg/ml)分别为 2 881± 16 5 3 ,988± 6 96 ,135 2± 413 ,15 40± 5 6 6 ,明显高于正常对照 (P <0 0 0 2 ) ,SLE病人sFas含量高于RA ,MS ,IDDM病人。SLE、RA患者血清sFasL含量 (pg/ml)分别为5 35± 431、12 38± 1184,明显高于正常对照 (P <0 0 2 ) ,MS、IDDM患者血清sFasL含量 (pg/ml)分别为 2 5 1± 140 ,2 11± 73 ,低于正常对照 (P >0 0 5 )。在SLE、RA病人中 ,高浓度sFasL者伴有高浓度sFas。在SLE病人中 ,所有抗ssDNA抗体阳性者均伴有高浓度sFas,所有抗sFas阴性者 ,ssDNA抗体也为阴性。结论　在SLE等疾病中sFas水平明显高于正常人 ,可作为疾病进展与治疗效果的判断指标。抗ssDNA抗体与sFas具有关联性。sFas与sFasL在疾病中的相互作用及动态变化有待进一步研究