抗磷脂抗体与抗磷脂血栓综合征

抗磷脂抗体（ＡＰＡ）与抗磷脂血栓综合征（ＡＰＬ－Ｔ）的发生密切相关,但ＡＰＡ引起ＡＰＴ－Ｔ的发病机制仍不清楚,近年来ＡＰＡ通过抑制蛋白Ｃ（ＰＣ）途径,引起获得抗活化蛋白Ｃ现象进而导致血栓的研究受到关注。本主要将抗磷脂血栓综合征的研究进展作一综述。     

抗磷脂抗体与抗磷脂血栓综合征

抗磷脂抗体(APA)与抗磷脂血栓综合征(APL-T)的发生密切相关,但APA引起APL-T的发病机制仍不清楚,近年来APA通过抑制蛋白C(PC)途径,引起获得性抗活化蛋白C现象进而导致血栓的研究受到关注。本文主要将抗磷脂血栓综合征的研究进展作一综述。     

抗心磷脂抗体检测和抗磷脂抗体综合征诊断

磷脂是指分子中含有醇 ,脂肪酸和磷酸基团的一类化合物。人体内的磷脂主要是含有甘油醇的甘油磷脂 ,包括心磷脂 ,磷脂酰丝氨酸 ,磷脂酰胆固醇 ,磷脂酰乙醇胺等。抗磷脂抗体 (antiphospholipidantibody ,aPL)是一族针对带负电荷磷脂或带负电荷磷脂与蛋白复合物的异质性抗体。抗磷脂抗体综合征 (antiphospholipidsyndrome ,APS) ,是一组与抗磷脂抗体有关的自身免疫性疾病 ,典型的临床表现有动脉血栓 ,静脉血栓以及妊娠丢失。APS患者血中检出aPL是确立APS诊断的必要条件。根据一些aPL可以识别磷脂或磷脂与蛋白复合物的特性 ,采用心磷脂包…     

抗凝血酶原抗体和抗磷脂抗体综合征

凝血酶原属于维生素K依赖性凝血因子。抗磷脂抗体综合征主要表现为血栓和反复流产。低亲和力的抗凝血酶原抗体与抗磷脂抗体综合征存在一定的联系。抗凝血酶原抗体检测标准化将有助于抗磷脂抗体综合征的诊断。     

抗磷脂抗体与血栓形成

抗磷脂抗体（包括狼疮样抗凝物及抗心磷脂抗体）是多种原因诱发的异质性自身抗体，进一步的研究表明抗磷脂抗体与血栓形成密切相关，检测抗磷脂抗体对临床医师预测，诊治血栓病具有一定的指导意义，本文综述了抗磷脂抗体的分类，与血栓形成的关系及检测方法，防治措施。     

抗磷脂抗体综合征的实验与临床

抗磷脂抗体（antiphospholipid antibody,APA）的本质仍不清楚,但大多学认为是一组酸性磷脂的异质性自身抗体的总称.它包括狼疮抗凝因子（lupus anticoagulant,LA）、抗心磷脂抗体（anticardiolipin antibodies,ACA）两大类,抗磷脂酸抗体、抗磷脂酰丝氨酸抗体、抗磷脂酰乙醇胺抗体、抗磷脂酰胆碱抗体、抗磷脂酰肌醇抗体等均可归于这两大类中.     

抗凝血酶原抗体和抗磷脂抗体综合征

凝血酶原属于维生素K依赖性凝血因子。抗磷脂抗体综合征主要表现为血栓和反复流产。低亲和力的抗凝血酶原抗体与抗磷脂抗体综合征存在一定的联系。抗凝血酶原抗体检测标准化将有助于抗磷脂抗体综合征的诊断     

抗磷脂综合征

近年来,一些学者发现系统性红斑狼疮(SLE)及某些自身免疫性疾病患者血清中存在抗磷脂抗体(APLA),包括狼疮抗凝物质(LA)和抗心磷脂(ACL).随着研究的深入,对APLA的临床意义获得了较多的认识,发现静脉和动脉血栓、神经精神症状、血小板减少,反复流产等临床表现与APLA密切相关,并提出血清中具有APLA的患者伴静脉和/或动脉血栓、血小板减少等表现者称抗磷脂综合征(APS).本文复习有关文献,对APS作一综述.一、APLA的生物学特性和发生率     

抗磷脂综合征血栓形成临床分析

抗磷脂综合征是一组自身免疫性疾病,其患体内抗磷脂抗体阳性,临床特点为血栓形成、反复流产,分为原发性抗磷脂综合征及继发性抗磷脂综合征.1995年至2003年,共收治抗磷脂综合征19例,就其血栓形成作一临床分析,报告如下.     

抗磷脂血栓综合征与获得性抗活化的影响C现象

了解抗磷脂抗体（ＡＰＡ）,抗活性化的蛋白Ｃ（ＡＰＣＲ）与抗磷脂血栓综合征（ＡＰＬ－Ｔ）的关系,进一步探讨ＡＰＬ－Ｔ血栓发生机制。方法以ＥＬＩＳＡ检测ＡＣＡ（ＩｇＭ,ＩｇＡ）;ＡＰＴＴ检测ＬＡ;ＡＰＴＴ＋／－ＡＰＣ检测ＡＰＣＲ。结果２０例患者符合ＡＰＬ－Ｔ诊断。根据病因分类,１００例ＳＬＥ患者中１４例为继发性ＡＰＬ－６：１６例“原因不明”血栓与习惯性流产患者中６例为原发性ＡＰＬ－Ｔ。根据抗体分类,２     

Antiphospholipid antibodies in arterial thrombosis

The high correlation between the IgG isotype of anticardiolipin antibodies (aCLs) and clinical thrombosis was first documented in 1983, and this observation was confirmed in subsequent studies. In addition, the frequency of fetal loss and thrombocytopenia was increased in this group of patients. These findings were termed the antiphospholipid syndrome (APS). This syndrome was mostly seen in patients with systemic lupus erythematosus (SLE), but it soon became clear that also other patients not suffering from defined SLE might exhibit features of APS. aCL in APS patients are detected in immunoassays by using solid phase cardiolipin as a putative antigen. However, antibodies directed against phospholipid-binding plasma or serum proteins, beta2-glycoprotein I (beta2-GPI), in particular, are also detected. Many recent studies have indicated that one of predominant antibodies that has been identified as aCL in APS patients is against beta2-GPI rather than any of the negatively charged phospholipids. The epitopes recognized by anti-beta2-GPI antibodies raised in APS patients are composed of discontinuous amino acid sequences from the IV domain of human beta2-GPI. These epitopes are cryptic when beta2-GPI does not interact with anionic phospholipids. An early event in atherosclerosis is the accumulation of cholesterol-laden foam cells, which originate mainly from monocyte-macrophage cells by their uptake of chemically modified low-density lipoprotein (LDL). We found that beta2-GPI binds directly to oxLDL, and that the complex of oxLDL and beta2-GPI is subsequently recognized by aCL (anti-beta2-GPI) to be taken up by macrophages. While the pathogenesis of this accelerated atherosclerosis is likely to be multifactorial, it is possible that antiphospholipid antibodies, including aCL (anti-beta2-GPI antibodies), may have contributed to the formation of atherosclerotic lesion.     

Antiphospholipid syndrome

Antiphospholipid syndrome has received considerable attention from the medical community because of its association with a number of serious clinical disorders, including arterial and venous thromboembolism, acute ischemic encephalopathy, recurrent pregnancy loss, thrombocytopenia, and livido reticularis. It can occur within the context of several diseases, mainly autoimmune disorders, and is then called secondary antiphospholipid syndrome. However, it may be also be present without any recognizable disease, or so-called primary antiphospholipid syndrome. There is no defined racial predominance for primary antiphospholipid syndrome, although a higher prevalence of systemic lupus erythematosus (SLE) occurs in African Americans and the Hispanic population. Multiple terms exist for this syndrome, some of which can be confusing. Lupus anticoagulant syndrome, for example, is a misleading term, because patients may not necessarily have SLE, and it is associated with thrombotic rather than hemorrhagic complications. To avoid further confusion, antiphospholipid syndrome is currently the preferred term for this clinical syndrome. Antiphospholipid antibodies are found in 1% to 5% of young healthy control subjects; however, the incidence increases with age and coexistent chronic disease. The syndrome occurs most commonly in young to middle-aged adults; however, it also can occur in children and the elderly. Among patients with SLE, the prevalence of antiphospholipid antibodies is high, ranging from 12% to 30% for anticardiolipin antibodies, and 15% to 34% for lupus anticoagulant antibodies. In general, anticardiolipin antibodies occur approximately five times more often then lupus anticoagulant in patients with antiphospholipid syndrome. This syndrome is the most common cause of acquired thrombophilia, associated with either venous or arterial thrombosis or both. It is characterized by the presence of antiphospholipid antibodies, recurrent arterial and venous thrombosis, and spontaneous abortion. Rarely, patients with antiphospholipid syndrome may have fulminate multiple organ failure, or catastrophic antiphospholipid syndrome. This is caused by widespread microthrombi in multiple vascular beds, and can be devastating. Patients with catastrophic antiphospholipid syndrome may have massive venous thromboembolism, along with respiratory failure, stroke, abnormal liver enzyme concentrations, renal impairment, adrenal insufficiency, and areas of cutaneous infarction. According to the international consensus statement, at least one clinical criterion (vascular thrombosis, pregnancy complications) and one laboratory criterion (lupus anticoagulant, antipcardiolipin antibodies) should be present for a diagnosis of antiphospholipid syndrome. The hallmark result from laboratory tests that defines antiphospholipid syndrome is the presence of antibodies or abnormalities in phospholipid-dependent tests of coagulation, such as dilute Russell viper venom time. There is no consensus for treatment among physicians. Overall, there is general agreement that patients with recurrent thrombotic episodes require life-long anticoagulation therapy and that those with recurrent spontaneous abortion require anticoagulation therapy and low- dose aspirin therapy during most of gestation. Prophylactic anticoagulation therapy is not justified in patients with high titer anticardiolipin antibodies with no history of thrombosis. However, if a history of recurrent deep vein thrombosis or pulmonary embolism is established, long-term anticoagulant therapy with international normalized ratio (INR) of approximately 3 is needed.     

Antiphospholipid syndrome

Antiphospholipid Syndrome (APS) was first described by Hughes and sometimes called as Hughes syndrome. Recent studies revealed that the antigen to anticardiolipin antibody (aCL) is not cardiolipin itself but co-factor beta 2-GPI which expresses its epitope when it combines cardiolipin or gets oxidized. Lupus Anticoagulant is now possibly considered as anti-prothrombin antibody. Livedo including Snedden syndrome, pulmonary hypertension and skin ulcer became considered as the part of symptoms of this disease. In ISAPA 1998, it is reported from several laboratories that IgA aCL is also pathogenic to thrombosis as well as IgG aCL. Atherosclerosis is also accelerated by aCL. Catastrophic APS is rare but fatal, reported 3 cases in Japan and 50 cases in the world.     

Antiphospholipid syndrome: review

Antiphospholipid syndrome spans many medical disciplines. Classic criteria include the presence of anticardiolipin antibody or lupus anticoagulant with typical complications of thrombosis or pregnancy loss. Other common associated manifestations include livedo reticularis, thrombocytopenia, valvular heart disease, and nephropathy with renal insufficiency, hypertension, and proteinuria. Treatment of serious complications with anticoagulation is standard; generally warfarin for thrombosis and aspirin/heparin for pregnancy prophylaxis. Detailed recommendations regarding precise intensity and duration of anticoagulation are still a subject of debate.     

Antiphospholipid antibody syndrome.

Autoimmune aPL are associated with a well-defined clinical syndrome of vascular thromboses, recurrent fetal loss, thrombocytopenia, livedo reticularis, and valvular and neurologic abnormalities. A clinical diagnosis of SLE need not be present, and aPL syndrome in the absence of other well-defined autoimmune disease is termed PAPS. A positive test for aPL is defined by enzyme-linked immunoassay (aCL) or by functional coagulation assay (LAC). Anticardiolipin antibody and LAC are similar but probably not identical antibodies. The false-positive test for syphilis is less closely associated with clinical complications than are aCL and LAC. The mechanism of action of aPL is not yet known, although many theories have been advanced. Recent identification of beta 2-glycoprotein I, a serum glycoprotein, as an aPL cofactor suggests that inhibition of this protein's anticoagulant activity may be important. Autoimmune aPL differ from infection-induced aPL in important antibody characteristics, including IgG subclass, light chain preference, antibody avidity, and cofactor requirement. Both recognize negatively charged phospholipids, but various physical characteristics of the phospholipids alter the recognition patterns. Treatment of the aPL syndrome is not well defined. Anticoagulation with heparin, coumadin, or aspirin are currently widely used. Although corticosteroid, immunosuppressive therapy, and plasmapheresis may be used for severe, fulminant thrombosis, the efficacy of this treatment has yet to be proved.