自身免疫性溶血性贫血研究进展

自身免疫性溶血性贫血（autoimmune hemolyticanemia，AIHA）是一组由自身产生的抗红细胞抗体所造成的溶血性贫血。虽然对AIHA的认识已经很久，但其病因和发病机制迄今仍未完全阐明，对不少病例的治疗仍很困难。现将近年来有关AIHA的一些研究进展介绍如下。     

自身免疫性溶血性贫血

自身免疫性溶血性贫血(简称AIHA)是一种后天获得性溶血性贫血,因患者异常免疫反应,使体内产生自身抗体附着红细胞膜表面或游离于血管中。使自己未经改变的正常红细胞破坏加速所致的一种溶血性贫血(简称溶贫)。这种溶贫按自身抗体的血清学和免疫化学特性可分为温抗体型AIHA(约80%),冷凝集病(约15%)和阵发性冷性血红蛋白尿(约5%)。通常不指明那一种抗体引起的AIHA即指温抗体型,是最常见的类     

自身免疫性溶血性贫血患者的不规则抗体检测

自身免疫性溶血性贫血(AIHA)是一组由于B淋巴细胞功能异常亢进,免疫功能紊乱产生抗自身红细胞的抗体,致使红细胞加速破坏而引起的贫血[1].虽然,并不积极主张AIHA患者输血治疗,但当贫血严重时,仍需要输血,而且此类患者反复输血极易产生不规则抗体,但由于该病的免疫血清血特点,不规则抗体被自体抗体所掩盖,使得不规则抗体被漏检,发生溶血性输血反应[2].因此血型鉴定、不规则抗体筛选、相容性配血试验对于AIHA患者就显得更为重要.尤其是不规则抗体筛选和鉴定对于反复输血的患者更为重要.     

不同输血方法在自身免疫性溶血性贫血患者中的应用分析

自身免疫性溶血性贫血病是一种常见的疾病,其产生原因是因为患者的免疫系统功能紊乱,患者体内的B淋巴细胞由于亢奋产生自身抗体和补体吸附于红细胞表面,这种抗体会对自身的红细胞产生抵抗作用,使红细胞破坏从而造成溶血性贫血.自身免疫性溶血性贫血的临床症状表现为畏寒,发热,黄疸,腰背酸痛等.     

自身免疫性溶血性贫血和自身免疫性胰腺炎患者的自身抗体

<正>自身免疫性溶血性贫血自身免疫性溶血性贫血是B淋巴细胞功能异常亢进,产生抗红细胞自身抗体,与红细胞膜表面的抗原结合,然后活化补体,激活巨噬细胞,使红细胞破坏加速;或是自身抗体促进补体与红细胞的结合,使红细胞的寿命缩短,从而引起获得性溶血性贫血的一组疾病。根据自身抗体与红细胞反应的最佳温度将自身免疫性溶血性贫血分为温抗体型和冷抗体型两大     

儿童平均红细胞体积增大的相关疾病分析

目的 探讨儿童平均红细胞体积>96fL的相关疾病.方法 在881例确诊的贫血患儿中共发现平均红细胞体积增大患儿96例,对该群患儿的平均红细胞体积和红细胞分布宽度等指数进行分析.结果 96例平均红细胞体积增大患儿中,营养性巨幼细胞性贫血2例(2.1%),非营养性巨幼细胞性贫血94例(97.9%),其中甲状腺功能减低症2例,化疗药物所致者4例,骨髓增生异常综合征(MDS)11例,自身免疫性溶血性贫血(AIHA)21例,再生障碍性贫血(AA)31例,白血病25例.营养性巨幼细胞性贫血、大部分MDS、AA、白血病和AIHA表现为红细胞大小不均一性贫血.以各种疾病间的发生率比较存在显著差异(χ2=60.862,P=0.000).结论 研究中发现收治的儿童大红细胞性贫血常见的疾病是MDS、白血病、AIHA、AA;营养性巨幼细胞性贫血所占比例较少.     

免疫性血液病治疗的药物选择

免疫性血液病应包括因自身免疫反应引起血细胞及造血组织损伤或其发病主要因免疫机制介导的血液疾病 ,该类疾病在治疗上的主要对策为免疫调整剂。1　自身免疫性溶血性贫血 (ＡＩＨＡ)ＡＩＨＡ系体内免疫调节功能紊乱 ,产生自身抗体和 /或补体 ,结合在红细胞膜上 ,导致红细胞破坏加速而引起的一组溶血性贫血。根据自身抗体作用的特点 ,分为温抗体及冷抗体。温抗体在 37℃时作用最活跃 ,以ＩｇＧ为主 ,为不完全抗体。冷抗体在2 0℃以下 ,作用较活跃 ,主要为ＩｇＭ ,为完全抗体。属于后者的为冷凝集素综合征 (ＣＡＳ)和阵发性寒冷性血红蛋白尿…     

Evans综合征15例临床分析

Evans综合征是一种自身免疫性疾病。1982年Pegels等证实患者血中存在自身红细胞及血小板抗体,使之破坏加速,临床表现为自身免疫性溶血性贫血(AIHA)和自身免疫性血小板减少性紫癜(AITP)。我院自1986年以来共收治15例,分析如下。     

自身免疫性溶血性贫血的诊断及现代治疗

自身免疫性溶血性贫血的诊断及现代治疗中国医科大学第一临床学院血液内科（１１０００１）肖卫国陈维信自身免疫性溶血性贫血（ＡＩＨＡ）是由于机体产生的自身抗体与患者红细胞表面抗原结合，或激活补体而发生的溶血性疾患。本病并非少见，国外统计约占溶血性疾患的１／...     

自身免疫性溶血性贫血患者成分输血的临床价值分析

目的分析研究自身免疫性溶血性贫血(AIHA)患者成分输血的临床价值。方法回顾性分析我院收治的42例自身免疫性溶血性贫血患者,给予患者实施成分输血,对其临床资料进行回顾性分析。结果 42例AIHA患者中,直接抗人体蛋白试验显示阳性34例(80.9%),合并间接抗人球试验阳性19例,15例患者采用成分输血联合血浆置换术治疗,12例成功治疗,3例治疗无效死亡。结论给予自身免疫性溶血性贫血患者采用洗涤红细胞输注合并血浆置换治疗,应用效果显著,值得应用。     

AIHA/Evans综合征患者外周血CD80、CD86及CD4^+CD25^+T细胞的表达及临床意义

目的探究自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)/Evans综合征患者外周血CD80、CD86及CD4^+CD25^+调节性T细胞的表达水平及其临床意义。方法选取2014年1月-2017年12月经南阳医学高等专科学校第一附属医院诊治的AIHA/Evans综合征患者60例,以是否处于发作期为准分为发作组与缓解组,其中发作组39例,缓解组21例,另同期随机选取28名健康志愿者作为对照组,比较三组外周血的淋巴细胞共刺激因子CD80、CD86及CD4^+CD25^+调节性T细胞的表达水平。结果AIHA/Evans综合征发作组CD80、CD86、CD4^+CD25^+调节性T细胞表达水平分别为(7.21±1.84)%、(6.23±4.42)%、(2.83±1.75)%,发作组CD80、CD86表达水平高于缓解组及对照组,CD4^+CD25^+调节性T细胞表达水平低于缓解组及对照组,差异均具有统计学意义(P<0.05),但缓解组与对照组CD80、CD86及CD4^+CD25^+调节性T细胞表达水平的比较差异无统计学意义。结论AIHA/Evans综合征发作患者外周血CD80、CD86及CD4^+CD25^+调节性T细胞的表达水平明显升高,CD80、CD86及CD4^+CD25^+调节性T细胞可能在AIHA/Evans综合征发病机制中起着重要作用。     

Rates of autoimmune diseases in Kaiser Permanente for use in vaccine adverse event safety studies

Safety monitoring following new vaccine introduction includes assessment of potential new onset autoimmune diseases (AID). As knowledge regarding AID background rates is limited, we evaluated the incidence of 11 AID in Northern California Kaiser Permanente. AID cases were identified using electronic records of members aged 10–62 years from 1998 to 2004, excluding those with AID diagnoses from 1996 to 1997. Using prespecified criteria, all identified cases of rare diseases were verified by medical record review, while a sample of cases was reviewed for common diseases; incidence rates were calculated based on the proportion of confirmed cases. Overall, the incidence of AID varied from 0.8/100,000 person-years (PY) for autoimmune hemolytic anemia (AIHA) to 54.1/100,000 PY for thyroiditis. Incidence rates in increasing order were AIHA, juvenile rheumatoid arthritis, Guillain-Barre Syndrome, idiopathic thromobocytopenia purpura, transverse myelitis, systemic lupus erythematosus, uveitis, multiple sclerosis, rheumatoid arthritis, Type 1 diabetes mellitus and thyroiditis; incidence rates also varied according to age and gender. These background incidence rates should prove useful for future observational vaccine safety studies and will help guide evaluation of potential vaccine AID events following introduction of new vaccines.     

巨噬细胞炎性蛋白-1α及其受体在自身免疫性溶血性贫血患者骨髓的表达及意义

目的　探讨骨髓巨核细胞巨噬细胞炎性蛋白 - 1α(MIP - 1α)及其受体CCR5的表达情况在自身免疫性溶血性贫血 (AIHA)的发病机制中所起的作用。方法　免疫组织化学法检测正常人、AIHA患者及其经激素治疗后骨髓片造血干细胞MIP - 1α及其受体CCR5的表达。正常人和患者骨髓造血干细胞原代培养后 ,收集上清液用ELISA测定MIP - 1α的分泌情况。结果　治疗前MIP - 1α及其受体CCR5表达明显高于治疗后及正常对照组。患者原代细胞培养上清液内MIP - 1α的含量明显高于正常对照组。结论　MIP - 1α及其受体CCR5表达的升高与AIHA的发病密切相关。     

Biomarkers of polycyclic aromatic hydrocarbon (PAH)-associated hemolytic anemia in oiled wildlife

Polycyclic aromatic hydrocarbons (PAHs) in crude oil cause a range of adverse effects in oiled seabirds, one of the most common being hemolytic anemia via oxidative attack of erythrocytes by PAH metabolites resulting in hemoglobin leakage and formation of Heinz bodies. In such cases, haptoglobin and ferritin are up-regulated to sequester free Hb and iron in the circulation. We investigated these plasma proteins as biomarkers of PAH-induced Heinz body hemolytic anemia in oiled seabirds. Concentration ranges of PAHs, HAP and FT in plasma samples were 10-184 ng/ml, 0-2.6 mg/ml and 0-7.6 ng/ml, respectively. Dose-response relationships between plasma PAH exposure and haptoglobin and ferritin (FT) were investigated, and evidence of erythrocyte Heinz body formation studied in 50 oiled common guillemots stranded on the Norfolk Wash coast (East England). Haptoglobin was negatively correlated, and FT was positively correlated with PAH exposure. Heinz bodies were also observed confirming the toxic mechanism causing hemolytic anemia and counts were positively correlated with exposure. Our results support the application of these complementary biomarkers to assess hemolytic effects of oiling in wildlife biomonitoring, which also discriminate the influence of hemolytic versus inflammatory effects in oiled guillemots.     

贫血患者血液检验中生化仪的临床应用价值分析

目的:分析在为贫血患者实施血液诊断的过程中生化仪的临床应用效果.方法:纳入2018年2月~2019年2月在本院接受贫血治疗的患者78例为本次实验研究的对象,所有患者均经过病理证实,运用生化仪为患者进行血液检验,将检验结果与病理结果进行对比,分析生化仪在贫血患者血液检验中的应用价值.结果:本次实验研究中共有78例患者,生化仪血液检验结果为贫血的有74例,其检验准确率为94.9%;其中溶血性贫血、再生障碍性贫血以及缺铁性贫血患者分别有15例、17例、42例,其与病理结果相比无明显差异(P>0.05).结论:在为贫血患者实施血液检验的过程中生化仪的运用具有着相对较高的检出率,其对溶血性贫血、再生障碍性贫血以及缺铁性贫血均有着相对较高的诊断准确率,可以将其作为贫血患者的理想血液检验方法.     

亚急性铅中毒致溶血性贫血一例

铅中毒多表现为神经、消化、血液系统损害,如神经衰弱、腹痛、腹胀、黄疸、小细胞低色素性贫血等。首都医科大学附属北京朝阳医院收治1例因服用偏方导致亚急性铅中毒致重度溶血性贫血的患者,患者主要表现为腹绞痛、便秘、溶血性贫血,给予驱铅治疗后病情缓解。     

ERYTHROCYTE ABNORMALITIES IN EXPERIMENTAL MAGNESIUM DEFICIENCY

Changes in erythrocyte membrane morphology have been found to accompany the hemolytic anemia of magnesium deficiency.     

Antibody-mediated cell-specific hematopoietic autoimmune disorders

Although it is clear that immunologic mechanisms play a significant role in the pathophysiology of many hematologic diseases, there are relative few situations where it is possible to gain a detailed understanding of immune damage in vivo in humans. Autoimmune hemolytic anemia, immune thrombocytopenia and immune neutropenia as antibody-mediated cell-specific disorders are of particular interest in this regard. Autoimmune hemolytic anemia represents a group of disorders in which individuals produce antibodies directed toward one or more of their own erythrocyte membrane antigens. This leads to destruction of the antibody-coated erythrocytes. The pathophysiology of the decreased erythrocyte survival has been examined with increasing sophistication for many years. This paper first discusses the underlying mechanisms responsible for autoimmune hemolytic anemias then consider immune thrombocytopenia and immune neutropenia.     

The Spectrum of Transfusion Reactions

Acute and delayed hemolytic transfusion reactions are of concern to clinicians in almost every field. The problems and complications of transfusion are numerous, involving decisions for or against transfusional therapy for such conditions as autoimmune hemolytic anemia, DIC, and coagulopathies. Management of infectious and noninfectious sequelae is stressed.     

Acute lymphoblastic leukemia in a patient with congenital hemolytic anemia: case report]

The authors present a case of a 13-year-old girl with a history of congenital hemolytic anemia (spherocytosis) who developed acute lymphoblastic leukaemia. She received treatment according to the ALL-BFM 91 protocol standard risk group. During maintenance therapy an aplastic crisis caused by Parvovirus B 19 infection had developed. Chemotherapy was stopped in September 1999, and the patient remained in complete remission. In January, 2000 the patient presented with jaundice, caused by a stone in the ductus choledochus. Cholecystectomy and splenectomy were performed, and the the girl became symptomfree. The authors review the most frequent complications of congenital hemolytic anemias (aplastic crisis, haemolytic crisis and cholelithiasis). The occurrence of acute lymphoblastic leukaemia in a patient with congenital hemolytic anemia has not been previously reported.