再生障碍性贫血免疫致病机制的研究进展

造血干细胞缺乏导致造血功能衰竭一直被认为是再生障碍性贫血 (ＡＡ)的主要病理机制。最初应用体外造血细胞集落形成证实ＡＡ患者的骨髓中造血细胞明显减少或缺如 ,ＣＤ34 细胞数和集落形成能力均明显低于正常对照组[1] 。然而 ,应用免疫抑制剂如抗胸腺细胞或淋巴细胞球蛋白 (ＡＴＧ或ＡＬＧ)以及环胞菌素Ａ (ＣｙＡ)治疗ＡＡ ,其疗效为 50 %～80 % [2 ,3] ,由此表明既使重型再生障碍性贫血(ＳＡＡ) ,其体内仍有少量造血干细胞的残留 ,且造血干细胞减少可能是免疫功能异常介导的细胞毒性作用所致[4 ,5] 。近年的研究表明 ,由Ｔ淋巴细胞介导…     

再生障碍性贫血的免疫细胞治疗研究

目的:探讨免疫细胞治疗再障的安全性和有效性。方法:33例再障患者接受静脉输注自体或供者提供经体外激活的免疫细胞,每周一二次,观察症状是否改善,输血依赖、血象、骨髓像、骨髓活检、存活状态来评价疗效。结果:33例患者中,27例(82%)有效,其中完全缓解12例(36%),部分缓解15例(45%),无反应6例(18%)。3年存活率为90%。结论:免疫细胞治疗方法对再生障碍性贫血是安全和有效的。     

病毒相关性再生障碍性贫血的研究进展

流行病学调查及大量实验研究表明，多种病毒感染与再生障碍性贫血（ＡＡ）发生有关，其中以肝炎病毒，细小病毒Ｂ１９，巨细胞病毒和ＥＢ病毒最为重要，病毒致ＡＡ的机制相当复杂，既有病毒本身对造血干细胞的直接破坏，又有病毒激发的免疫损伤，此外，有的病毒还可能因破坏骨髓的基质细胞，破坏骨髓环境而影响骨髓的造血功能。     

间充质干细胞在再生障碍性贫血治疗中的应用

背景：造血干细胞移植现在仍然是儿童和青年重型再生障碍性贫血的一线治疗选择,但供者来源有限,费用高,且5%~15%的患者因为慢性移植物抗宿主病影响疗效和生活质量。因此尚需探索能提高疗效,减轻不良反应的治疗途径。间充质干细胞易于分离并且增殖力稳定,可以满足临床需求。 目的：总结间充质干细胞的研究进展、免疫学特性以及在再生障碍性贫血治疗中的应用情况。 方法：由第一作者在2010-06检索PubMed数据库(http://www.ncbi.nlm.nih.gov/PubMed)及中国期刊全文数据库(http://www.cnki.net)1995/2010有关间充质干细胞的研究进展、免疫学特性以及在再生障碍性贫血治疗中应用的文献。英文检索词为“mesenchymal stem cells,aplastic anemia,anemia”,中文检索词为“间充质干细胞,再生障碍性贫血,贫血”。 结果与结论：共收集119篇关于间充质干细胞及再生障碍性贫血的文献,中文85篇,英文34篇,排除因研究目的与课题无关、发表较早、重复及类似研究,纳入30 篇符合标准的文献。目前普遍认为再生障碍性贫血是一种获得性骨髓造血功能衰竭症,再生障碍性贫血的发生、发展与T细胞功能亢进引起的造血组织损伤密切相关。间充质干细胞具有免疫负调节及支持造血双重作用,且具有可移植性和来源广泛的特点。再生障碍性贫血患者和正常人的间充质干细胞在生物学特性上具有差异性,间充质干细胞将会为研究再生障碍性贫血的发病机制和治疗提供新的思路和方法。     

再生障碍性贫血的研究

近二十年,再生障碍性贫血(AA)的研究取得重大突破.中国AA年发病率约0.73/10万,病毒感染等危险因素与AA发病明显相关.AA已由"骨髓衰竭综合征"逐步被认知为自身免疫性T细胞功能异常相关的骨髓衰竭症.诊断AA关键依据骨髓造血功能低下、T细胞功能亢进及除外检查.治疗AA以强化免疫抑制和促造血为主且疗程必须充分.AA是可以治愈的.     

纯红细胞再生障碍和再生障碍性贫血的免疫抑制治疗

目的 观察免疫抑制剂对纯红细胞再生障碍（PRCA）、重型再生障碍性贫血（SAA）与非重型再生障碍性贫血（NSAA）的治疗作用。方法 采用小剂量环孢素A（3－5mg/kg.d）治疗7例PRCA、4SAA及4例NSAA,动态监测环孢素A的血药浓度。从外周血象的变化,来判断环孢素A的临床疗效。结果 环孢素A治疗3个月后,除1例SAA外,其余14例患者的血红蛋白水平均有较大的提高。随着治疗时间的延长,7例SAA与NSAA的白细胞数、中性粒细胞绝对数及血小板数也呈逐渐上升趋势。结论 环孢素A治疗PRCA、SAA及部分NSAA的近期效果良好。     

再生障碍性贫血患者细胞因子水平观察

为进一步探讨细胞免疫对ＡＡ发病的影响，阐明细胞因子在ＡＡ患者中变化的基础与临床意义，采用酶联免疫试剂盒ＥＬＩＳＡ法对３８例ＡＡ患者及２０例正常人外周血单个核细胞（ＰＢＭＮＣ）培养上清诱生Ｇ－ＣＳＦ，ＩＬ－６，ＴＮＦα，ＩＦＮα及ＩＬ－８水平进行测定，同时采用改良ＡＰＡＡＰ法观察外周血Ｔ细胞亚群及ＨＬＡ－ＤＲ抗原表达，结果ＡＡ患者ＰＢＭＮＣ培养上清中Ｇ－ＣＳＦ阳性率减低，ＩＬ－６，ＴＮＦαＩＦＮα及     

再生障碍性贫血患者TH水平的观察

近年来，许多学者认为甲状腺激素（Ｔｈｙｒｏｉｄ ｈｏｒｍｏｎｅ，ＴＨ）降低与病种无关，但常能反映疾病的严重程度［１］。本文对３５例再生障碍性贫血（再障）患者进行ＴＨ水平测定，旨在观察ＴＨ水平在再障中的动态变化。现将结果报告如下。 对象和方法 一、对象：再障组３５例（男２０，女１９），年龄１３－６３岁，为住本院的再障患者，其中急性再障５例，慢性再障３０例，均符合１９８７年第四届全国再生障碍性贫血学术会议修订的诊断标准［２］。对照组３０例（男１６，女１４），年龄１９－５８岁，系健康体检者。 二、方法：采…     

再生障碍性贫血患者甲状腺激素的RIA及其意义

我们对44例再生障碍性贫血（再障）患者进行了甲状腺激素的放射免疫分析（RIA）,现将结果报告如下。 对象和方法 一、对象: （一）正常人:选自本院健康体检合格者,无肝、肾、甲状腺病史者34例（男19,女15）。年龄18～47岁,平均36.5岁。 （二）再障患者:为本院住院患者44例（男23,女21）。诊断按1987年6月宝鸡会议所修订的标准。本组患者既往均无甲状腺病史。年龄8～65岁,平均31.7岁。 二、方法:空腹静脉采血分离血清,置-20℃备用。T_3、T_4、TSH、rT_3放免试剂盒由北方所提供。FT_3、FT_4放免试剂盒由中科院上海原子核研究所提供。     

Antibodies in the Treatment of Aplastic Anemia

Antibodies have been the cornerstone of treatment of acquired aplastic anemia for more than 25 years. Treatment with antithymocyte globulin (ATG) is considered pivotal and the addition of cyclosporine improves the overall response rate. This antibody is heterogeneous and horse ATG is apparently more effective than rabbit ATG. Several issues remain unsolved in relation to the combination of ATG and cyclosporine: cost, toxicity and late clonal disorders. In recent years, alternative immunosuppressive therapy has been proposed and new antibodies have emerged: porcine ATG, alemtuzumab, daclizumab, and rituximab. Experience with these antibodies is limited to a few studies with alemtuzumab being the most promising, but the results are interesting and provocative. More studies are needed to find the perfect antibody.     

坐骨神经损伤修复机制及其治疗的研究进展

坐骨神经损伤是坐骨神经干或分支受到外界创伤而引起的躯体感觉、运动及自主神经功能障碍的一种临床综合症。坐骨神经损伤修复的机制复杂,对其修复机制的深入研究将有利于提高本病的诊疗水平。本文就近年来坐骨神经损伤修复机制及其治疗方面的进展作一综述。     

再生障碍性贫血甲襞微循环研究

目的：探讨再生障碍性贫血（再障）甲襞微循环变化规律及其与临床的关系。方法：用WX－753B型微循环显微镜和WIS－Ⅱ型血流测速仪，对65例再障患者进行了观测分析。结果：再障甲襞微循环管襻形态、血液流态、襻周状态和总积分值均显著高于正常对照组；重型再障上述各积分值均显著高于慢性再障；肾阴虚型甲襞微循环总积分值最高，肾阴阳两虚次之，肾阳虚最轻；证型转化时甲襞微循环也随之变化；再障甲襞微循环异常程度与外周血红蛋白浓度、血小板计数呈显著负相关。结论：再障患者甲襞微循环异常程度与中西医分型、病情变化、外周血细胞数、药物疗效等密切相关。     

Treatment of aplastic anemia with antilymphocyte globulin and methylprednisolone with or without cyclosporine. The German Aplastic Anemia Study Group

BACKGROUND AND METHODS. Immunosuppression is the most effective treatment for patients with aplastic anemia, except for bone marrow transplantation. The best results are achieved with antilymphocyte globulin or cyclosporine. Patients have been treated successfully with a combination of both agents, but there has been no controlled evaluation of its efficacy. We conducted a randomized, multicenter trial in 84 patients not eligible for bone marrow transplantation, comparing treatment with antilymphocyte globulin and methylprednisolone (41 patients--the control group) with antilymphocyte globulin, methylprednisolone, and cyclosporine (43 patients--the cyclosporine group). RESULTS. At three months significantly more patients in the cyclosporine group had a complete or partial remission in response to treatment than did patients in the control group (65 percent vs. 39 percent, P less than 0.03); this difference was confirmed at six months (70 percent vs. 46 percent, P less than 0.05). The superior results of the regimen including cyclosporine were most evident in the patients with severe or very severe aplastic anemia, whose response rate at six months was 65 percent, as compared with 31 percent of such patients in the control group (P less than 0.02). Granulocyte and hemoglobin levels became normal in most patients who responded, but platelet counts continued to be subnormal in 61 percent of the patients. Ten of 52 patients with responses (3 in the cyclosporine group and 7 in the control group) relapsed 4 to 37 months after treatment. The actuarial survival of all patients at 41 months is 64 percent in the cyclosporine group and 58 percent in the control group (P = 0.16); among the patients with severe or very severe disease, survival is 80 percent and 44 percent, respectively (P = 0.077). Cyclosporine had substantial but reversible side effects. CONCLUSIONS. Immunosuppressive treatment of aplastic anemia with antilymphocyte globulin, methylprednisolone, and cyclosporine appears to be more effective than a regimen of antilymphocyte globulin and methylprednisolone without cyclosporine and may thus represent a treatment of choice for patients who are not eligible for bone marrow transplantation.     

膝关节骨性关节炎的发病因素及治疗进展研究

膝关节骨性关节炎是一种老年性关节炎,会引起多种临床症状及并发症,对患者的生活带来严重的影响。本文通过对膝关节骨性关节炎的发病因素分析,从非药物治疗、药物治疗以及手术治疗分别讲述各自的特点,适用情况,为膝关节骨性关节炎的发病因素的临床治疗提供理论依据。     

Allogeneic Hematopoietic Stem Cell Transplantation for the Treatment of Severe Aplastic Anemia Patients with Infection: A Single-Center Retrospective Study

To assess the efficacy of allogeneic hematopoietic stem cell transplantation (allo-HSCT) for severe aplastic anemia (SAA) patients with infection, we conducted a retrospective study on 65 SAA patients with infection who received allo-HSCT from August 2012 to December 2016. All patients received antibacterial and/or antifungal therapy before transplantation. The infection status after initial anti-infection therapy was classified as complete response (CR) (n?=?14) or partial response/stable disease (PR/SD) (n?=?51) before transplantation. The median times for myeloid engraftment in the PR/SD and CR groups were 10.5 days (range, 7 to 22) and 10 days (range, 8 to 11), with cumulative incidences of 98% and 100%, respectively. With a median follow-up of 788 days (range, 181 to 1758), patients with PR/SD had comparable results for 3-year estimated overall survival (85.4% versus 92.9%, P?=?.530) and 3-year failure-free survival (82.7% versus 92.9%, P?=?.458) with 14 patients with CR who received contemporaneous transplantation. In multivariate analysis, poor survival outcomes for the entire cohort was significantly associated with poor pretransplantation performance status. This retrospective study indicated that allo-HSCT may be a feasible therapeutic option for SAA patients with infection.     

32例再生障碍性贫血的微循环及血液粘度

对３２例再障患者的甲襞、球结膜微循环及血粘度进行观察，结果表明：再障组均有不同程度的微循环异常改变（１００％），与正常对照组比较差异显著（Ｐ＜０．０１）；再障组全血粘度、全血还原粘度明显低于正常对照组（Ｐ＜０．０１），再障患者的Ｈｂ与全血粘度及血浆比粘度呈正相关。     

重型再生障碍性贫血伴肛周脓肿患者的护理体会

目的：探讨对重型再生障碍性贫血伴肛周脓肿患者的护理体会。方法对1例重型再生障碍性贫血伴肛周脓肿的患者实施止血、抗感染以及纠正贫血和肛周特殊护理。结果经过治疗和护理,患者生命体征平稳,肛周疼痛好转,肛周脓肿处破皮,引流通畅、周围皮肤未见发红。结论对重型再障伴肛周脓肿患者,采取有效的对症支持治疗,能充分减低患者的痛苦,降低患者的经济费用,也提高了患者的满意度。     

再生障碍性贫血患者细胞免疫指标的观察

采用酶免疫和放射免疫法分析了再生障碍性贫血(AA)患者外周血T淋巴细胞亚群、血清肿瘤坏死因子(TNF)、γ-干扰素(IFN-γ)和IL-2水平,以及患者外周血单个核细胞体外诱生的上述细胞因子水平。结果表明:患者CD4/CD8细胞比值降低或倒置(P<0.001),血清TNF和IFN-γ水平显著增高(P<0.01),外周血单个核细胞诱生的TNF、IFN-γ和IL-2水平均明显高于正常对照(P<0.01)。提示T淋巴细胞亚群比例失调和TNF、IFN-γ等造血负调控因子的过量分泌可能与AA的发病机制有关。