抗磷脂抗体综合征

抗磷脂抗体(Anti-phospholipid antibody APLA)的研究是一个新的领域,其意义仅在八十年代初才逐渐被认识。很多研究证实特异性的APLA与某些临床表现有关,尤其与反复的动脉或静脉血栓形成,血小板减少及习惯性流产关系密切。APLA包括梅毒血清反应假阳性(BFPSTS),循环狼疮抗凝质(Lupus-1ike anticoagulant LA),以及与心磷脂及其他负电荷磷脂交互反应的抗体。APLA还可以引起其他许多临床表现,包括皮肤损害,如皮肤溃疡,网状青斑;神经系统病变,如缺血性脑卒中,一过性脑缺血发作,偏头痛和午蹈病等;视网膜的动脉和静脉血栓;以及酷似Libman-Sacks心内膜炎的心脏损害。血管病理表现为大小动脉、静脉闭塞及血栓形成。     

特发性血小板减少性紫癜患者抗心磷脂抗体的临床意义

探讨IgG型抗心磷脂抗体（IgG－ACLA）在特发性血小板减少紫癜（ITP）患者中的临床意义。ITP患者血清IgG－ACLA阳性率不低，临床应引起重视，警惕抗磷脂综合征的可能，且在ITP患者ACLA和血小板相关抗体可同时存在。     

抗磷脂抗体阳性导致反复自然流产的诊治心得

抗磷脂综合征(APS)是由抗磷脂抗体引起，主要表现为反复性自然流产、血栓形成、血小板减少等的一组临床症候群。     

抗磷脂综合征对妊娠的影响与治疗

抗磷脂综合征（antiphospholipidsyndrome，APS）是一种自身免疫性疾病，由抗磷脂抗体引起，其临床特点为反复动静脉血栓形成和产科并发症。后者主要包括复发性妊娠丢失、胎儿生长受限、胎盘功能不全、妊娠高血压疾病等。患者血液中出现多种针对带负电荷的磷脂的自身免疫抗体，即抗磷脂抗体（APA），主要包括狼疮抗磷物（LA）、抗心磷脂抗体（ACA）、抗磷脂酰丝氨酸抗体（aPS）、抗磷脂酰肌醇抗体（aPI）和抗磷脂酸抗体（aPA）等。目前APS成为复发性自然流产及病理产科的主要免疫因素，是近年来产科研究的热点。抗磷脂抗体阳性者若不予治疗，自然流产率高达50％～90％。因此，这类妇女在计划怀孕前，应到医院就诊，进行病因筛查，阳性者进行积极治疗，可明显提高妊娠成功率。笔者就APS对妊娠的影响及其治疗进展作一综述。     

神经梅毒误诊为抗磷脂抗体 综合征7例临床分析

神经梅毒（neurosyphilis,NS）是由苍白密螺旋体 （treponema pallidum）侵犯脑膜和（或）脑实质引起的 一种慢性中枢神经系统感染性疾病[1],多经性行为传 播。有资料显示,近年来神经梅毒的发病率显著上 升[2],因其临床表现复杂多样,起病隐匿,极易引起误 诊。抗磷脂抗体综合征（antiphospholipid syndrome, APS）是由抗磷脂抗体,包括狼疮抗凝物（lupus anti? coagulants,LA）和抗心磷脂抗体（anticaidiolipin anti? bodies,ACA）所引起的一种自身免疫性疾病,临床上 以静脉或动静脉血栓形成、习惯性流产及血小板减 少症为其主要表现[3]。临床上,根据有无合并其他自 身免疫性疾病,将 APS 分为原发性抗磷脂综合征 （primary antiphcpholopid syndrome,PAPS）和继发性 抗磷脂综合征（secondary antiphcpholopid syndrome, SAPS）。现对我院收治的误诊为抗磷脂抗体综合征 的7例神经梅毒患者的临床资料进行分析,以提高对 神经梅毒的认识,减少误诊。     

磷脂相关抗体诱发血小板活化的机制研究进展

抗磷脂综合征（APS）是以抗磷脂抗体持续阳性为实验室标准,血栓形成或病理妊娠为临床特征的自身免疫性疾病。APS 发病机制尚未阐明,“二次打击”学说指出血小板、内皮细胞的重要性,血小板活化作为 APS 征血栓性事件的主要触发机制获得广泛认可。本文就 APS 患者血栓事件形成中抗 β2 糖蛋白Ⅰ抗体、抗心磷脂抗体、狼疮抗凝物等抗磷脂抗体与血小板活化关系的研究进展进行综述。     

血栓性抗磷脂综合征的治疗进展

抗磷脂综合征（antiphospholipid syndrome,APS）是一种全身性的自身免疫性疾病,以血管血栓形成或妊娠并发症为特征,伴有持续阳性的抗磷脂抗体（aPL）,包括狼疮抗凝物（LA）,抗心磷脂抗体（aCL）和抗β2-糖蛋白I（β2GPI）抗体。目前血栓性APS的治疗包括血栓事件的一级及二级预防、复发血栓、产科并发症以及灾难性抗磷脂综合征的治疗。本文对血栓性APS治疗方案的更新及主要研究进展进行综述。     

抗心磷脂抗体在病理妊娠中的意义

目的：探讨抗心磷脂抗体与病理妊娠的关系。方法：对447例行抗心磷脂抗体金标免疫斑点定性试验的病人及孕产妇行对照研究。结果：①各病理妊娠组ACA阳性率明显高于正常妊娠组,中期流产、胎膜早破;妊娠合并肝内胆汁淤积症;胎儿宫内发育迟缓;死胎、早产;妊娠合并血小板减少的阳性率明显高于正常妊娠组,且统计学上有显著差异（P＜0．05）,而妊娠高血压综合征、胎儿畸形、妊娠剧吐、妊娠合并乙肝较正常对照无统计学意义（P＞0．05）。②研究抗心磷脂抗体与既往流产史的关系发现有流产史者较无流产史者显著增高。（P＜0．05）。结论：①抗心磷脂抗体阳性与流产、胎膜早破、早产、死胎、胎儿宫内窘迫有关。②有流产史者抗心磷脂抗体可增高。③抗心磷脂抗体阳性与妊娠高血压综合征的发病可能无关。     

抗心磷脂抗体与儿童特发性血小板减少性紫癜的关系

抗心磷脂抗体与儿童特发性血小板减少性紫癜的关系四川省人民医院（６１００７２）李戈，郭晋蓉近年来研究发现２８～３１％的ＩＴＰ患者有血清抗心磷脂抗体（ＡＣＡ）存在’‘·’‘。这些抗体可与血小板膜上的磷脂结合，增加了网状内皮系统对血小板的摄取和破坏，导致血...     

抗磷脂综合征相关急性脑梗死的特点

抗磷脂综合征（anti—phospholipid syndrome,APS）是一种非炎症性自身免疫病,临床上表现为反复发生的动、静脉血栓形成（包括脑血栓、脑静脉窦血栓、深静脉血栓、冠状动脉血栓、外周动脉血栓、习惯性流产和血小板减少等症状,血清中存在抗磷脂抗体（antiphospholipid antibody,APLA）。上述症状可以单独存在,也可多个共存。     

Antiphospholipid antibody-related Bell's palsy in young women

The present report describes three young women aged 25, 20 and 15 years who presented with Bell's palsy. Two of the patients had a past history of the disease, which responded to steroid treatment. All three patients were positive for antiphospholipid antibody (APLA). In addition, one of the patients tested positive for antinuclear antibodies; however, there was no clinical evidence of systemic lupus erythematosus. All three patients responded well to steroid therapy. We conclude that Bell's palsy may be one of the manifestations of APLA syndrome, and thus, APLA testing should be done in such cases.     

Delayed-onset heparin-induced thrombocytopenia. A potentially malignant syndrome

Delayed-onset thrombocytopenia developed in 12 patients while they were receiving either prophylactic or therapeutic heparin. Five of the patients had thrombocytopenia alone, and seven had thromboembolic complications which contributed to the death of one patient. These complications included deep venous thrombosis (four patients), pulmonary embolism (three patients), myocardial infarction (one patient), sagittal sinus thrombosis (one patient), and femoral artery occlusion (one patient). The diagnosis of heparin-induced thrombocytopenia was delayed for between one and 13 days after the initial complicating event. All patients had heparin-dependent platelet-aggregating factor in their plasma. The characteristics of the heparin-dependent platelet-aggregating reaction were the same in all patients, but the nadir of thrombocytopenia was lower in patients with delayed-onset heparin-induced thrombocytopenia and complicating thromboembolism. These findings highlight the necessity for early recognition of this syndrome and for the prompt withdrawal of heparin to prevent considerable patient morbidity.     

抗磷脂综合征患者血栓事件的危险因素分析

目的：探讨影响抗磷脂综合征（antiphospholipid syndrome, APS）患者血栓事件发生的危险因素。方法：回顾性分析北京大学人民医院收治的61例APS患者血栓事件的发生特点,评价血栓事件与患者临床及免疫学指标的相关性,包括性别、年龄、有无血小板减少、吸烟、高血压、糖尿病、高脂血症和抗磷脂抗体等。结果：70.49% APS患者发生血栓事件,其中动脉血栓发生率36.67%,静脉血栓发生率39.34%,下肢静脉血栓和脑梗塞最常见,分别占37.7%和24.59%,其次为肺栓塞、血栓性微血管病、肾梗死和脾梗死。抗心磷脂抗体阳性和高血压分别是静脉和动脉血栓事件的独立危险因素。结论：抗心磷脂抗体阳性者易发生静脉血栓,而合并高血压的APS患者易发生动脉血栓。     

Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating lupus anticoagulant

The lupus anticoagulant was found in the plasma of 31 of 60 patients with systemic lupus erythematosus and other connective tissue disorders (mixed connective tissue disease, systemic vasculitis, polyarteritis nodosa, primary sicca syndrome, discoid lupus, Behcet's syndrome, and systemic sclerosis). Strong associations were found with biological false positive seroreaction for syphilis and thrombocytopenia. The most striking association, however, was with the high prevalence of thrombosis. This tendency to thrombosis was independent of disease activity of systemic lupus erythematosus. The lupus anticoagulant appears to be a useful marker for a subset of patients with systemic lupus erythematosus at risk for the development of thromboembolic complications.     

Bad Obstetric History: A Prospective Study

Background

Death of an infant in utero or at birth has always been a devastating experience for the mother and of concern in clinical practice. Perinatal mortality remains a challenge in the care of pregnant women worldwide, particularly for those who had history of adverse outcome in previous pregnancies. To assess the risk factors and outcome of pregnancies in cases of bad obstetric history (BOH) and compare the results with control group, this study was undertaken.

Methods

A prospective study from 2003 to 2007 was carried out in 79 pregnancies having BOH (history of unexplained stillbirth/neonatal death, three or more consecutive abortions etc). Test group was analyzed in terms of age, gravida, parity, risk factors and outcome in terms of preterm delivery, stillbirth, mode of delivery, birth weight, pregnancy complications and fetal distress. These parameters were compared with a systematic, randomly selected sample from rest of the deliveries. Necessary advice and treatment was given in cases of hypothyroidism, hypertension, antiphospholipid antibody (APLA) syndrome, gestational diabetes and other risk factors.

Result

There was significantly higher incidence of malpresentations, hypertension, APLA, cervical incompetence, preterm deliveries and caesarean section in test group (p< 0.05). In this study, only 47 (59.49%) women out of 79 in BOH group were identified to have possible factor responsible for pregnancy losses. In 32 (40.51%), no probable causes could be identified. Nine (11.39%) patients were identified with more than one risk factor.

Conclusion

APLA, hypertension, malpresentation, cervical incompetence, preterm deliveries and caesarean section were found significantly more in BOH group. In a large percentage of pregnancies with BOH, the risk factors for adverse outcome were not identified but pregnancy outcome was generally good in subsequent pregnancies with optimal antenatal care and advice.Key Words: Bad obstetric history, Antiphospholipid antibody syndrome, Gestational diabetes, Stillbirth     

原发免疫性血小板减少症发生血栓的影响因素研究

背景原发免疫性血小板减少症（ITP）是以血小板减少为特征的出血性疾病，但其血栓事件发生情况却高于正常人群，进而增加了其治疗难度，因此分析其血栓发生的危险因素非常必要。目的探究ITP患者发生血栓的相关影响因素。方法选取2010年1月至2021年6月于商丘市第一人民医院住院的725例ITP患者为研究对象，依据患者是否形成血栓分为血栓组及非血栓组。回顾性收集患者的一般资料、抗磷脂抗体检测结果及血栓类型。采用多因素Logistic回归分析探究ITP患者发生血栓的影响因素。结果725例ITP患者中非血栓组704例（97.1%）、血栓组21例（2.9%）。血栓组患者年龄≥50岁、有高血压史、有糖尿病史、激素使用>8周、脾切除、狼疮抗凝物（LA）阳性、抗心磷脂抗体（aCL）阳性所占比例高于非血栓组（P<0.05）；血栓组与非血栓组患者疾病状态情况比较，差异有统计学意义（P<0.05）。多因素Logistic回归分析结果显示，年龄〔≥50岁：OR=4.466，95%CI（1.008，19.797），P=0.049）〕、疾病状态〔慢性ITP：OR=3.046，95%CI（1.067，8.699），P=0.037）〕、高血压史〔有：OR=9.031，95%CI（1.930，42.272），P=0.005〕、糖尿病史〔有：OR=5.919，95%CI（2.078，16.863），P=0.001〕、激素使用>8周〔是：OR=4.119，95%CI（1.465，11.580），P=0.007〕、LA〔阳性：OR=3.426，95%CI（1.145，10.254），P=0.028）〕、aCL〔阳性：OR=4.064，95%CI（1.357，12.169），P=0.021〕是ITP患者发生血栓的影响因素（P<0.05）。725例ITP患者中发生动脉血栓12例（1.7%）、静脉血栓8例（1.1%）、形成动静脉血栓1例（0.1%）。结论年龄≥50岁、疾病状态为慢性ITP、有高血压史、有糖尿病史、激素使用>8周、LA阳性、aCL阳性是ITP患者发生血栓的影响因素，临床治疗ITP时应特别注意预防其血栓的发生。     

109例妊娠期血小板减少的临床分析

 目的 探讨妊娠期发生血小板减少的病因、临床特征及其对母婴的影响,降低围生期母婴出血并发症的发生。方法 回顾性分析复旦大学附属妇产科医院2001年11月至2008年6月收治的109例妊娠期伴有血小板减少的孕产妇临床资料。结果 多数患者诊断为妊娠相关性血小板减少（pregnancy-associated thrombocytopenia,PAT）,21例（19.3%）诊断为免疫性血小板减少性紫癜（idiopathic thrombocytopenic purpura,ITP）,其中妊娠合并ITP 20例、妊娠合并Evans’综合征 1例;5例（4.6%）诊断为妊娠高血压综合征者,其中1例为先兆子。新生儿发生血小板减少者5例（5.9%）。分娩时出血量和血小板下降的程度无明显关联。结论 妊娠期出现血小板减少的病因有多种,以PAT和ITP最多见。既往有血小板减少、过敏、病毒感染、自身免疫性疾病等病史均是妊娠期发生血小板减少的高危因素。     

Successful Treatment of Severe Heparin-induced Thrombocytopenia with Intravenous Immunoglobulin,Platelet Transfusion and Rivaroxaban: A Case Report

Heparin-induced thrombocytopenia (HIT) is a relatively infrequent complication of heparin administration. HIT can cause devastating thrombosis, making it one of the most serious adverse drug reactions encountered in clinical practice. We successfully treated a case of severe HIT presenting with thrombosis and life-threatening bleeding complications with intravenous immunoglobulin (IVIG), platelet transfusion and oral anticoagulant Rivaroxaban. In this case, we considered that IVIG played the most important role by preventing further thrombosis, increasing the platelet count, and ensuring the efficacy of Rivaroxaban. We therefore suggest that IVIG might be the optimal treatment for patients with this urgent condition.     

抗心磷脂抗体与手术后深静脉血栓形成

目的 探讨抗心磷脂抗体（ACL抗体）在术后深静脉血栓形成（DVT）中的临床意义。方法 通过酶联免疫吸附法检测42例DVT患者的ACL抗体，其中22例为术后DVT患者，20例为无诱因DVT患者；另以44例健康体检者为对照组。结果 术后DVT患者的ACL抗体阳性率为27.3%，无诱因DVT患者的ACL抗体阳性率为55.0%，两者与正常对照组比较，差别均有显著性意义（P＜0.05及0.01）；在DVT患者中，ACL抗体与自发性流产和血小板减少症有明显相关性（均P＜0.05）。结论 对术前有血栓形成史、自发性流产史和不明原因血小板减少的患者有必要检测ACL抗体，阳性者术中、术后应给予抗凝剂以预防DVT的发生。     

抗磷脂血栓综合征

目的:了解抗磷脂抗体(APA)与抗磷脂血栓综合征(APL-T)相关性,进一步探讨APL-T血栓发生机制。方法:采用 ELISA检测ACA(IgG,M,A),PTT-LA检测LA,APTT /-APC检测APC-R。结果:20例患者符合 APL-T诊断。根据病因分类:14/100例SLE为继发性 APL-T;6/16例“原因不明”血栓与习惯性流产为原发性APL-T。根据抗体分类:12/20例LA阳性为狼疮抗凝因子血栓综合征(LA-T);12/20例ACA阳性为抗心磷脂抗体血栓综合征(ACA-T)。根据临床症状分类:7/20例静脉血栓为Ⅰ型,3/20例脑梗塞为Ⅱ型;1/20例静脉血栓合并脑梗塞,习惯性流产为Ⅳ型(混全型);9/20例习惯性流产为Ⅴ型APL-T。APL-T患者LA阳性率(12/20,60％)明显高于正常对照组(NC组0/20,0%)。患者ACA-IgG,M与NC组比较有显著性差异(P<0.05);APCR在APL-T患者中阳性率(14/20,70％)明显高于NC组(l/20,5％);LA阳性组中的APCR阳性率(10/11,90.9％)明显高于LA阴性组(l/11,9％),ACA阳性组的APCR阳性率(7/11,63.3％)与ACA阴性组(4/11,36.36％)比较有显著性差异。结论:APA与APCR密切相关,APL-T中血栓发生率与APA以及APCR存在明显相关性,说明APA对PC途径的抑制引起获得性APCR可能是APL-T血栓发生的重要原因,故可将APA作为血栓危险性增加的标志物。