促血小板生成素及其抗体在系统性红斑狼疮伴发血小板减少中的作用

目的探讨促血小板生成因子(TPO)和抗TPO抗体在系统性红斑狼疮(SLE)患者血小板减少中的作用。方法于2016年3月至2017年5月纳入在四川大学华西医院、川北医学院附属医院、新疆自治区人民医院、四川省简阳市人民医院风湿免疫科住院治疗的SLE伴血小板减少患者50例和SLE血小板正常患者43例,同期选择基线资料匹配的健康体检者27人。采用ELISA法测定受检者血清TPO水平和抗TPO抗体滴度,评估TPO在SLE患者血小板减少中的作用,同时评估受检者血清TPO水平和抗TPO抗体滴度与血小板计数变化的关系。采用MMT法测定抗TPO抗体对体外培养的巨核细胞增生的影响。结果正常对照组、SLE血小板正常组和SLE伴血小板减少组受检者血清TPO质量浓度分别为(63. 7±20. 6)、(98. 5±28. 7)和(80. 0±24. 8) pg/ml,SLE血小板正常组患者血清TPO水平明显高于SLE伴血小板减少组和正常对照组,差异均有统计学意义(P=0. 003、0. 002)。正常对照组、SLE血小板正常组和SLE伴血小板减少组血清抗TPO抗体滴度分别为0. 476±0. 165、0. 679±0. 300和0. 763±0. 348,SLE血小板正常组和SLE伴血小板减少组患者抗TPO抗体滴度明显高于正常对照组,差异有统计学意义(P=0. 000、0. 007)。SLE伴血小板减少组患者血小板计数与血清抗TPO抗体滴度呈明显负相关(r=-0. 268,P=0. 048),与血清TPO水平无线性相关(r=0. 133,P=0. 393)。SLE伴血小板减少组和SLE血小板正常组受检者血清抗TPO抗体阳性率分别为32. 0%(16/50)和25. 6%(11/43),SLE伴血小板减少组与SLE血小板正常组患者血清抗TPO抗体阳性率差异无统计学意义(χ2=0. 462,P=0. 497)。MTT试验结果显示,随着抗TPO抗体滴度增加,TPO诱导的巨核细胞的增生活力逐渐下降,差异均有统计学意义(均P0. 01)。结论 SLE患者血清可检测出抗TPO抗体,在TPO分泌相对不足的情况下,抗TPO抗体通过抑制巨核细胞增生而在SLE伴发血小板减少的过程中发挥作用。     

淋巴细胞对系统性红斑狼疮患者粒巨噬细胞集落形成单位抑制活性的研究

目的　探讨系统性红斑狼疮 (SLE)患者B、T淋巴细胞对SLE及正常人骨髓粒巨噬细胞集落形成单位 (GM CFU)的影响。方法　分别用SLE患者及正常人B、T及B +T淋巴细胞加入半固体甲基纤维素培养体系中 ,观察它们分别对 16例SLE患者及 12名正常人GM CFU产率的影响。结果　加入SLE患者B、T淋巴细胞后 ,分别可见 75 0 %和 37 5 %的SLE患者GM CFU形成减少 ,产率为 (10 9± 71)个 /10 5细胞和 (137± 34 )个 /10 5细胞 ,与未加淋巴细胞组比较差异均有显著性(P <0 0 1,P <0 0 5 ) ,同时加入SLE患者B和T淋巴细胞时 87 5 %的SLE患者GM CFU产率减少 ,为 (98± 31)个 /10 5细胞 ,与分别加入SLE患者B或T淋巴细胞组比较均有明显差异 (P <0 0 5 ,P <0 0 1) ;SLE患者B及B +T淋巴细胞分别对 5 8 4%、6 6 7%的正常人骨髓GM CFU形成有抑制作用 ,产率为 (2 0 3± 6 7)、(199± 92 )个 /10 5细胞 ,明显低于未加淋巴细胞组 (P均 <0 0 1) ,而正常人淋巴细胞对SLE患者GM CFU的产率与未加淋巴细胞组比较差异无显著性 (P均 >0 0 1)。结论　SLE患者B淋巴细胞无论对大部分SLE患者或大部分正常人骨髓GM CFU的形成有抑制作用 ,SLE患者的B +T淋巴细胞对SLE患者和正常人骨髓GM CFU的抑制有协同作用 ,SLE患者的B淋巴细胞的异常可能是SL     

系统性红斑狼疮病人甘露糖结合凝集素基因多态性和血清水平的研究

目的探讨系统性红斑狼疮(SLE)病人甘露糖结合凝集素(MBL)基因多态性及血清水平与疾病的关系。方法用聚合酶链反应-限制性片段长度多态性(PCR-RFLP)法检测44例SLE病人和45名正常人MBL外显子1的第54密码子的多态性(GGC/GAC),同时用酶联免疫吸附试验(ELISA)检测血清中MBL的含量。结果SLE病人GGC/GGC型71%(31/44),GGC/GAC型27%(12/44),GAC/GAC型2%(1/44);正常人MBL基因型分布频率GGC/GAC型78%(35/45),GGC/GAC型22%(10/45);两组相比,分布差异无显著性。不同基因型SLE病人血清MBL水平分别为(1.8±0.6)mg/L(31例),(1.5±0.6)mg/L(12例),1例GAC/GAC型血清MBL水平为1.17mg/L;正常人血清MBL水平分别为(3.0±1.0)mg/L(35例),(2.2±1.0)mg/L(10例)。正常人GGC/GGC型和GGC/GAC型血清MBL水平均明显高于SLE病人组(P<0.01,P<0.05)。结论SLE病人的血清MBL水平明显减低,但似与基因第54号密码子变异无相关性。     

系统性红斑狼疮合并重症血小板减少的临床特征

目的　研究系统性红斑狼疮 (SLE)并发重症血小板减少的临床特征 ,并与慢性特发性血小板减少性紫癜 (CITP)急性发作进行对比。方法　比较SLE并发重症血小板减少组 (SLE组 )和CITP组病人的出血情况、血小板相关抗体、骨髓巨核细胞成熟障碍程度、对泼尼松(≥ 1mg·kg-1·d-1)联合长春新碱近期治疗效果等的差异。结果　SLE组出血程度轻 ,以皮肤、黏膜出血为主 ,而CITP组出血程度重 ,以黏膜和脏器出血为主 ;两组间血小板相关抗体差异无显著性 (P>0 0 5 ) ;SLE组骨髓巨核细胞总数显著低于CITP组 (P <0 0 5 ) ,巨核细胞成熟被阻碍在颗粒巨核细胞阶段的程度低于CITP组 ;SLE组泼尼松 (≥ 1mg·kg-1·d-1)联合长春新碱的近期疗效明显低于CITP组 (P <0 0 5 )。结论　SLE并发重症血小板减少在骨髓巨核细胞成熟障碍的程度、出血程度及对治疗的反应等方面与CITP均有显著不同之处     

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     

再生障碍性贫血和特发性血小板减少性紫癜患者血小板生成素的检测及意义

目的测定再生障碍性贫血(AA)和特发性血小板减少性紫癜(ITP)二组血小板减少患者血小板生成素(TPO)质量浓度并探讨其临床意义.方法采用ELISA法测定13例初治AA和20例初治ITP患者血清TPO质量浓度,以17例健康体检者为正常对照.结果正常对照组血清TPO质量浓度为(99.41±73.84)ng/L,AA患者血清TPO质量浓度为(683.48±414.73)ng/L,明显高于正常对照组(P＜0.01);ITP患者血清TPO质量浓度为(100.67±79.59)ng/L,与正常对照组相比,差异无显著性(P＞0.05).相关分析表明AA患者TPO质量浓度与血小板计数呈负相关(r=-0.71,P＜0.01);而ITP患者TPO质量浓度与血小板计数无相关性(P＞0.05).结论血清TPO的质量浓度受循环中血小板数目和骨髓巨核细胞总体数量的双重调节.由于血小板生成减少所致的血小板减少症其血清TPO质量浓度明显升高;而由于血小板破坏过多所致者其血清TPO质量浓度处于正常水平.     

CD154在活动期系统性红斑狼疮B淋巴细胞中的表达

目的探讨CD154在系统性红斑狼疮(SLE)中的异常表达及其在狼疮发病中的作用机制。方法16例活动期SLE及14名正常健康人,分离外周血CD19阳性B细胞,以流式细胞仪荧光抗体标记检测其CD154的表达,并体外观察抗CD154抗体对外周血B细胞的增生及IgG分泌的影响。结果①活动期SLE外周血B淋巴细胞中CD154阳性率(36±17)%及表达强度(364±238)均显著高于正常健康人,后者分别为(10±8)%,124±97%(P均<0.001);②体外单独培养,活动期SLE外周血B淋巴细胞自身即可异常增生并分泌IgG,[3H]-TdR掺入及体外培养上清液IgG浓度与正常人相比,差异有显著性(P值分别<0.0001和0.001)。抗CD154抗体可显著抑制活动期SLE外周血B淋巴细胞的异常增生及IgG的异常分泌,[3H]TdR掺入及体外培养上清液IgG浓度与对照抗体组相比,差异有显著性(P值分别为0.027和0.034)。结论CD154在活动期SLE的B淋巴细胞中有异常表达,其异常调控可能是导致分泌自身抗体B细胞克隆增生的主要原因。     

系统性红斑狼疮患者血清中B淋巴细胞刺激因子的检测

目的检测系统性红斑狼疮(SLE)患者血清B淋巴细胞刺激因子(BLyS)水平,并探讨其在SLE发病中的意义。方法采用双抗体夹心酶联免疫吸附试验(ELISA)法检测血清BLyS水平。结果①SLE患者血清BLyS[(9.6±2.3)ng/ml]显著高于正常人对照组[(4.0±1.5)ng/ml]。②SLE患者中,血清BLyS水平活动组[(11.1±2.2)ng/ml]高于非活动组[(8.1±1.2)ng/ml],抗dsDNA抗体阳性组[(10.9±2.2)ng/ml]高于抗dsDNA抗体阴性组[(8.1±1.4)ng/ml],高IgG组[(10.8±2.4)ng/ml]高于非高IgG组[(8.3±1.3)ng/ml],低C3组[(10.2±2.5)ng/ml]高于非低C3组[(8.3±1.3)ng/ml],低C4组[(10.1±2.3)ng/ml]高于非低C4组[(7.6±0.7)ng/ml],低血小板计数组[(10.7±2.7)ng/ml]高于非低血小板计数组[(8.8±1.7)ng/ml]。③SLE患者血清BLyS水平与SLE疾病活动指数(SLEDAI)(r=0.56,t=15.89,P<0.01)、IgG(r=0.33,t=4.20,P<0.05)呈正相关;与C4(r=-0.47,t=10.04,P<0.01)、血小板计数(r=-0.53,t=13.85,P<0.01)呈负相关。结论BLyS可能参与SLE的发病。     

伴血细胞减少系统性红斑狼疮34例骨髓细胞形态学分析

目的 了解伴血细胞减少的系统性红斑狼疮(SLE)骨髓细胞形态学变化.方法 对34例外周血细胞减少的SLE患者(WBC<4×109/L, 或Hb<100 g/L, 或Pt<100×109/L)的骨髓涂片进行回顾性细胞形态学检查,并与26例特发性血小板减少性紫癜(ITP)、18例骨髓增生异常综合征的难治性贫血(MDS-RA)患者以及14例健康对照者综合分析.结果 34例SLE患者中24例(70.6%)骨髓发育不良,包括红系多核(三核或更多)10例(29.4%),巨幼样变8例(23.5%), Pelger-Hüet样畸形12例(35.3%),多核巨核细胞8例(23.5%),小型巨核细胞10例(29.4%). 结论在伴血细胞减少的SLE患者中能观察到骨髓多系细胞发育不良,这些改变说明骨髓可能是SLE侵犯的靶器官之一.     

系统性红斑狼疮患者骨髓CD34+细胞表面标志及与病情活动的相关性分析

目的研究系统性红斑狼疮(SLE)患者骨髓CD34+细胞表面标志的变化,了解SLE患者造血干细胞是否存在异常。方法应用流式细胞术CD45/SSC设门分析10例SLE患者和10例正常人骨髓CD34+细胞CD90、CD117、CD123、CD164、CD166、CD95(FAS)、FAS-L、人类白细胞抗原(HLA)-DR等表面分子的表达及其与病情活动指标的相关性。结果活动期SLE患者骨髓CD34+细胞比例(1.5±0.4)%,明显低于正常人(2.3±0.8)%,P<0.01;非活动期患者(2.0±0.4)%与正常人相比差异无统计学意义。SLE患者CD34+、CD95+的表达明显高于正常人[(48.3±10.6)%vs(24.3±11.1)%,P<0.05],患者CDl23和CDl66也明显高于正常人[(45±22)%vs(20±4)%,P<0.05];[(31±20)%vs(11±6)%,P<0.05]。其余表面标志的表达与正常人相比差异无统计学意义。CDl23表达率与患者外周血白细胞计数负相关(r=-0.700,P< 0.05),与SLE疾病活动指数(SLEDAI)评分无相关性。CD166表达与SLEDAI(r=0.472,P<0.05),血清C3 (r=-0.712,P<0.01),尿蛋白定量(24h)(r=0.558,P<0.05)显著相关。结论SLE患者骨髓CD34+细胞CD95、CDl23、CDl66的表达率增加,CDl23的表达率与外周血白细胞计数显著负相关:CDl66的表达率与SLEDAI评分、24h尿蛋白呈显著正相关,与血清C3呈显著负相关,CDl66可能是一个新的SLE疾病活动性标志。     

A patient with rectal ulcer with severe stenosis presenting with perforated peritonitis

We report a patient with rectal ulcer with severe stenosis, who underwent urgent surgical treatment for perforated peritonitis. The 54-year-old man suddenly developed cramping abdominal pain and fever while hospitalized, with signs of peritoneal irritation. An emergency laparotomy was performed, and severe stenosis of the rectum and a perforated lesion on the oral side approximately 10 cm distant from the stenosis were found, with massive abdominal purulent fluid. He was treated by rectosigmoid colon resection with transverse colon loop colostomy. Histopathologically, the stenosis was caused by ulceration extending to all muscular layers of the rectum, with inflammatory changes. Benign rectal stenosis is so rare that differential diagnosis from malignancy may be difficult when there are inflammatory changes in the surrounding tissues. However, it is necessary to keep in mind the likelihood of this disease in differentiation from rectal cancer. Received: December 21, 1998 / Accepted: May 28, 1999     

肿瘤病人弓形虫感染分析

在肿瘤的发生和发展进程中 ,多伴有免疫功能低下或缺陷 ,从而极易遭受各种感染。弓形虫是机会感染因子 ,当患者免疫功能受损时 ,易于感染 ,还会使隐性感染激活 ,引起低热不退、淋巴结肿和脑神经系统的反应 ,此现象尚未引起临床医师的重视。近年来 ,我们对 4 0 9例肿瘤病人进行了弓形虫感染及弓形虫病的分析观察 ,报告如下 :1　材料与方法1 1　材料　 30 4例病人血清取自江西省肿瘤医院住院或门诊病人 ,随机抽样后低温保存待检 ,10 5例取自其他医院送检样品 ,有急性症状者随到随检 ,以便及时做病原学检测。1 2　弓形虫病诊断方法1 2 1　免疫…     

Evaluation of atrial vulnerability with transoesophageal stimulation in patients with atrioventricular junctional: Comparison with patients with ventricular pre-excitation and with normal subjects

The aim of our work was to evaluate the inducibility of atrialfibrillation in a group of patients with atrioventricular junctionalreentrant tachycardia and to compare it with that of patientswith a Kent-type ventricular pre-excitation (Wolff-Parkinson-Whitesyndrome) and a control group. One hundred and twenty-five subjects were separated into groups.Group 1 comprised 49 Wolff-Parkinson-White patients, with amean age of 26.4, range 10.66 years; group 2, 51 patients withatrioventricular junctional reentrant tachycardia inducibleby transoesophageal atrial stimulation andlor clinically documented,with a mean age of 43.4, range 16–78 years; group 3, 25control subjects with a mean age of2.64, range 13–76 years. Each subject underwent atrial transoesophageal stimulation withthe following protocol: programmed atrial stimulation with 1and 2 stimuli during atrial pacing of 100. min^–1 and 150.min^–1; atrial stimulation for 10 s at a rate of 200–300–400–500–600.min^–1 with intervals of 10 s between stimulations, fivesuccessive ‘ramp-up’ atrial stimulations for 9 swith the rate increasing from 100 to 800. min^–1 with intervalsof 10 s between stimulations. The end point was the completionof the protocol or induction of sustained atrial fibrillation(>1 min). The chi-square test was used for statistical analysis. Our resultsshowed that in group 1 atrial fibrillation was induced in 27149patients (55.1%); this was sustained in 13149 (26.5%) and non-sustainedin 14149 (28.5%); in group 2, atrial fibrillation was inducedin 22151 patients (43.0%); it was sustained in 7151 (13.7%)and non-sustained in 15151 (29.4%); in group 3, sustained atrialfibrillation was not induced in any subject and in only onesubject was a non-sustained atrial fibrillation (4 s) induced. The chi-square test showed that group 2 vs group 1 were non-significant,while group 2 vs group 3 and group 1 vs group 3 were significant(P<0.003 and P<0.0007, respectively). Therefore group 2 patients showed a greater atrial vulnerabilityin comparison to the control subjects and a similar vulnerabilityto group 1 patients. It is possible that the greater atrialvulnerability in the patients of group 2 was due to the doublenodal pathway.     

Infection with Rhodococcus equi in a patient with sarcoidosis treated with corticosteroids

A 51-year-old female farmer was diagnosed as having sarcoidosis. During 4 years of observation, slow radiological progression was observed. Cough then developed, necessitating treatment with corticosteroids. After 28 months of continuous treatment with prednisolone in low doses (5-7.5 mg daily), she suffered fever episodes, recurrent haemoptyses, general malaise and loss of weight. A chest roentgenogram showed a left upper lobe infiltrate, which progressed and finally cavitated, and rib destruction. Despite efforts, including a thoracotomy, 22 months passed before a diagnosis could be made. Blood and sputum cultures and cultures from the destroyed rib showed growth of Rhodococcus equi, a common soil organism which can cause infections in foals and other animals. Treatment with rifampicin and erythromycin was successful. R. equi has been reported to cause infection in patients with neoplastic disease and/or immunosuppression, but the disease might be more common than is suggested by the sparse case reports in the literature, owing to lack of familiarity with the organism, which will tend to be overlooked as a contaminant.     

Treating patients with lupus with B-cell depletion

A new era in the treatment of systemic lupus erythematosus has dawned with the increasing introduction of monoclonal antibodies and other approaches, that target the key molecules involved in the pathogenesis of the disease. At present the ability to block the CD20 molecule on those B cells that carry this marker has proved the most effective way to treat patients resistant to conventional immunosuppressive drugs. However, these studies have all been open label and the results of double blind controlled studies are eagerly awaited.