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关键词： 固有淋巴细胞   癌症   细胞因子  

摘要： ILC作为组织中存在的一类能对周围环境变化产生迅速反应的免疫细胞,近年来一直是研究的热点,并在癌症的发生进展和转移中发挥不可替代的作用。文章综述了在不同癌症类型中,不同ILC亚群及其相关细胞因子通过与肿瘤微环境相互作用,发挥促进或抑制肿瘤的效应,以全面阐释ILC在癌症中的影响。

关键词： Cronkhite-Canada综合征   IgG4   IgE   免疫紊乱  

摘要： 目的探讨Cronkhite-Canada综合征(Cronkhite-Canada syndrome,CCS)患者血清免疫球蛋白特征及其临床价值。方法纳入2009年12月至2024年9月于北京协和医院收治并进行血清免疫球蛋白检测的CCS患者,回顾性分析其临床特点及辅助检查结果。结果共纳入52例患者,男36例(69.2%),女16例(30.8%),中位起病年龄60(54~64)岁,起病至诊断的中位时间为5(3~12)个月。常见临床表现包括腹泻(86.5%)、腹痛(40.4%)、便血(19.2%)、体重下降(86.5%)、指甲营养不良(100.0%)、色素沉着(88.5%)、毛发脱落(84.6%)、味觉减退(69.2%)等。21例(50.0%)患者存在血清IgG下降,20例(71.4%)的患者存在总IgE升高。11例(23.4%)血清IgG4高于参考值,IgG4升高组与IgG4正常组间的临床指标无显著差异,活动期CCS患者(n=43)与缓解期患者(n=27)的IgG4水平比较差异无统计学意义。总IgE升高组的毛发脱落率显著高于IgE正常组(P=0.0383)。结论CCS患者存在外周血免疫球蛋白紊乱,其中IgE升高与毛发脱落有关,提示CCS患者的毛发脱落可能与免疫介导机制相关。

关键词： 三金片   急性尿路感染   p38MAPK/NF-κB信号通路   炎症因子   紧密连接蛋白  

摘要： 目的:通过观察实验性急性尿路感染小鼠膀胱组织显微结构、炎症评分、免疫组化以及血清炎症指标等改变,探讨三金片治疗急性尿路感染小鼠的作用机制。方法:采用经典Murine急性尿路感染动物造模方法制作急性尿路感染小鼠模型,分成正常组、模型组、三金片组,每组10只。造模成功后,正常组、模型组均给予0.9%无菌氯化钠溶液灌胃,三金片组予等量药物无菌0.9%氯化钠溶液药液灌胃,每日1次,连续给药5d。干预5d后,观察膀胱组织病理及超微结构;检测膀胱组织、血清中的炎症指标,膀胱组织p38MAPK、NF-κB蛋白及Occludin、Claudin-2蛋白进行作用机制探析。结果:药物干预5d后,光镜下,相较于模型组,三金片组小鼠膀胱组织黏膜病理上皮炎症细胞、水肿、间质增生、血管增生、充血减轻,移行上皮层数减少;透射电镜下,相较于模型组,三金片组小鼠膀胱组织细胞排列更紧密,细胞核形态较完整,线粒体更丰富,可见溶酶体,且固有肌层平滑肌排列较整齐,未见明显炎细胞浸润和血管增生。与模型组比较,三金片组膀胱组织促炎因子IL-1β降低(P<0.01),p38MAPK蛋白相对表达量下降(P<0.05);血清促炎因子IL-1β呈现降低趋势;三金片组膀胱组织NF-κB蛋白相对表达量以及Occludin、Claudin-2蛋白相对表达量呈上升趋势。结论:三金片能改善急性尿路感染膀胱组织炎症情况及修复细胞微结构,降低促炎因子IL-1β介导p38MAPK控制炎症。

关键词： 骨髓增殖性肿瘤   WT1   BCR-ABL   白细胞介素-6   血管内皮生长因子   血浆蛋白C   血浆蛋白S   相关性  

摘要： 目的探讨骨髓增殖性肿瘤(MPN)患者WT1、BCR-ABL基因与白细胞介素-6(IL-6)、血管内皮生长因子(VEGF)、血浆蛋白C(PC)、血浆蛋白S(PS)的相关性。方法选取2021年1月到2024年1月于该院接受治疗的54例MPN患者作为MPN组,另选取同期进行体检的54例健康者作为对照组。检测并比较两组WT1、BCR-ABL基因相对表达水平和IL-6、VEGF、PC、PS水平,分析WT1、BCR-ABL基因相对表达水平与IL-6、VEGF、PC、PS水平的相关性,受试者工作特征(ROC)曲线分析WT1、BCR-ABL基因对MPN的诊断效能。结果与对照组比较,MPN组WT1、BCR-ABL基因相对表达水平和IL-6、VEGF水平更高,PC、PS水平更低,差异有统计学意义(P<0.05)。Pearson相关分析结果显示,WT1、BCR-ABL基因相对表达水平与IL-6、VEGF水平呈正相关,与PC、PS水平呈负相关(P<0.05)。WT1、BCR-ABL基因二者联合较单独指标诊断MPN的效能高,曲线下面积(AUC)为0.833,灵敏度为88.69%。结论MPN患者WT1、BCR-ABL基因相对表达水平与IL-6、VEGF、PC、PS水平存在相关性,WT1联合BCR-ABL基因可有效诊断MPN。

关键词： 新生儿感染性肺炎   静注人免疫球蛋白(pH4)   注射用头孢哌酮钠舒巴坦钠   炎症因子  

摘要： 目的分析新生儿感染性肺炎(NIP)行静注人免疫球蛋白(pH4)联合注射用头孢哌酮钠舒巴坦钠治疗的临床效果。方法选取138例NIP患儿,以随机数字表法分为对照组和观察组,各69例。对照组给予注射用头孢哌酮钠舒巴坦钠治疗,观察组在对照组基础上加用静注人免疫球蛋白(pH4)治疗。对比两组疗效、症状缓解时间、免疫功能指标、炎症因子指标、不良反应发生情况。结果观察组总有效率95.65%高于对照组的75.36%(P<0.05)。观察组肺啰音消失时间、喘息消失时间、咳嗽消失时间、呼吸困难缓解时间、体温稳定时间分别为(5.05±0.49)、(2.15±0.24)、(5.06±0.82)、(2.81±0.28)、(2.42±0.32)d,均短于对照组的(6.47±0.78)、(3.08±0.38)、(7.11±1.23)、(3.54±0.43)、(3.46±0.43)d(P<0.05)。治疗后,观察组CD4^(+)、CD4^(+)/CD8^(+)、免疫球蛋白G(IgG)、IgG1、IgG2、IgG3水平均高于对照组,CD8^(+)水平低于对照组(P<0.05)。治疗后,观察组白细胞介素-6(IL-6)、降钙素原(PCT)、C反应蛋白(CRP)水平均低于对照组(P<0.05)。两组不良反应发生率对比无明显差异(P>0.05)。结论NIP治疗时选择注射用头孢哌酮钠舒巴坦钠联合静注人免疫球蛋白(pH4)疗效显著,且具较高安全性,可采纳。

关键词： 多发性骨髓瘤   沙利度胺   免疫球蛋白   肾功能损伤  

摘要： 目的:观察沙利度胺辅助治疗多发性骨髓瘤的效果。方法:选取盐城市大丰人民医院血液科2021年1月—2024年6月收治的65例多发性骨髓瘤患者为研究对象,随机分为对照组(n=30)及观察组(n=35)。对照组接受硼替佐米联合地塞米松方案治疗,观察组在对照组方案基础上联用沙利度胺。比较两组治疗效果、免疫球蛋白水平及肾功能损伤指标水平。结果:观察组治疗有效率高于对照组(P=0.013);治疗后,观察组免疫球蛋白A、免疫球蛋白G水平低于对照组(P<0.001);治疗后,观察组血清肌酐、血尿素氮、尿β2微球蛋白水平低于对照组(P<0.001)。结论:沙利度胺辅助治疗多发性骨髓瘤的效果显著,可改善患者免疫球蛋白水平并减轻肾功能损伤。

关键词： Atypicaldiverse phenotype   Gene therapy   IL2RG   Self-inactivating lentiviral vector   X-linked severe combined immunodeficiency disease  

摘要： X-linked severe combined immunodeficiency disease(X-SCID)is a rare inheriteddisease caused by mutations in the interleukin 2 receptor subunit gamma gene(IL2RG),whichencodes the common g chain protein,a subunit of the receptor for lymphocytes.X-SCID ischaracterized by profound defects in T-cell,B-cell,and natural killer cell ***,we report a Chinese cohort of nine X-SCID patients with six novel IL2RG ***,the two adolescent patients with an atypical immunotype were confirmed by furtheranalyzing IL-2-JAK-STAT5 signaling,T cell proliferation,and T cell receptor excision circles(Trecs).Interestingly,Bacillus Calmette-Guerin(BCG)disease occurred commonly in *** allogeneic hematopoietic stem-cell transplantation is curative for the disease,it is not available to all patients due to the lack of suitable matched *** therapy using a self-inactivating lentiviral vector(SIN-LV)technology has provided analternative therapy for such mono-genetic ***,we performed the pre-clinicalstudies to assess our SIN-LV carrying IL2RG on human ED7R cells deficient in IL2RG andCD34^(+)stem cells derived from the bone marrow of a healthy donor and a patient with *** work is done complied with the established“Good Manufacturing Practice”(GMP)used inthe clinical *** addition,a safety study is performed using the transduced CD34^(+)cells implanted into the axilla of nude mice in ***,our studies have demonstrated the efficiency and safety of SIN-IL2RG-LV,which paves the way for conducting X-SCID gene therapyclinical trials in China in the near future.

关键词： ACR20/50/70   American College of Rheumatology response criteria   bDMARDs   biological Disease-Modifying Anti-Rheumatic drugs   cDAPSA   clinical disease activity index of psoriatic arthritis   DAPSA   disease activity index of psoriatic arthritis   DAS28-CRP   disease activity score based on 28 joints and C-reactive protein concentrations   EQ-5D   EuroQol group questionnaire in 5 dimensions   HR   hazard ratio   i   inhibitor   IL   interleukin   IPW   inverse propensity score-weighted   MASES   Maastricht ankylosing spondylitis enthesitis score   MDA   minimal disease activity   PS   propensity score   PsA   psoriatic arthritis   SCQM   Swiss Clinical Quality Management in Rheumatic Diseases   TC   treatment course   Th17i   inhibitors of the Th17 cell generation or function   TNF   tumor necrosis factor  

摘要： Objective To compare the effectiveness of Interleukin (IL)-12/23 or IL-17A inhibitors (summarized to inhibitors of the Th17 cell generation or function, Th17i) with tumor necrosis factor inhibitors (TNFi) in patients with TNFi-experienced psoriatic arthritis (PsA). Patients and Methods We conducted a comparative effectiveness study by taking advantage of prospectively collected patients with PsA data from the Swiss Clinical Quality Management in Rheumatic Diseases register, encompassing the interval from January 1, 2015 to August 1, 2021. Drug retention was the primary outcome in unadjusted and inverse propensity-weighted Cox regression models. Secondary outcomes were a static skin score for psoriasis, the American College of Rheumatology (ACR) 20, 50, and 70 response rates, and the disease activity in PsA score. Results At baseline, Th17i (n=341) were initiated in patients with more severe skin disease, but with comparable disease activity as TNFi (n=503) in all other disease domains. In the unadjusted analysis, Th17i were later discontinued than TNFi (median 828 vs 445 days, P <.001), but the hazard ratio for discontinuation was significantly lower for Th17i than for TNFi only in women (0.57 [0.37-0.87], P =.01). Furthermore, differences in static skin scores between the groups at baseline were equalized at follow-up. However, improvements in the disease activity in PsA were similar in both groups, and ACR20 (33% [29%] vs 14% [13%]; P =.03) and ACR50 response rates (24% [21%] vs 7% [6%]; P =.02) were even higher for TNFi in unadjusted and (LUNDEX-adjusted) analyses. Conclusion After TNFi failure, more profound skin improvement and longer drug retention in women argue in favor of switching to Th17i in certain patient populations. However, TNFi may at least be equivalent in improving locomotor system manifestations and remain a viable option in the first and in the later treatment line of PsA.

关键词： 阵发性睡眠性血红蛋白尿   再生障碍性贫血   牙源性感染   发热   罕见病  

摘要： 一例青年男性患者诊断再生障碍性贫血伴阵发性睡眠性血红蛋白尿(paroxysmal nocturnal hemoglobinuria,PNH),经免疫抑制剂治疗后仅部分缓解。近1年贫血加重、输血依赖,考虑PNH活动性溶血所致,更换依库珠单抗治疗后贫血仍持续加重。详细询问病史发现患者慢性牙周感染,近1个月牙周疼痛溢脓伴发热。多学科讨论考虑慢性牙源性感染激活补体系统是PNH溶血加重的主要原因,急性感染影响了依库珠单抗的疗效。经多学科会诊制定治疗方案,给予静脉抗生素联合牙周冲洗、拔牙控制牙源性感染,同时继续按期输注依库珠单抗,患者临床症状好转,血红蛋白迅速上升,脱离血制品输注。该病例为通过多学科协作诊疗解决罕见病在新药物治疗中遇到的困难提供了经验参考。