Finazzi G
Divisione di Ematologia, Ospedali Riuniti, Bergamo, Italy.
Haematologica. 1997 Jan-Feb;82(1):101-5.
The clinical importance of antiphospholipid antibodies (APA) derives from their association with a syndrome of venous and arterial thrombosis, recurrent fetal loss and thrombocytopenia known as the antiphospholipid syndrome (APS). The Italian Registry of Antiphospholipid Antibodies was set up in 1989 for the purpose of collecting a large number of patients with lupus anticoagulant (LA) or anticardiolipin antibodies (ACA) for clinical studies in order to obtain more information on the clinical features of APS.
The Italian Registry has completed two clinical studies and proposed an international trial on the treatment of APS patients. These activities of the Registry are reviewed herein. Additional information has been obtained from pertinent articles and abstracts published in journals covered by the Science Citation Index and Medline.
The first study of the Registry was a retrospective analysis of enrolled patients which showed that: a) the prevalence of thrombosis and thrombocytopenia was similar in cases with idiopathic APA or APA secondary to systemic lupus erythematosus, and b) the rate of thrombosis was significantly reduced in patients with severe thrombocytopenia but not in those with only a mild reduction of the platelet count. The second study was a prospective survey of the natural history of the disease, showing that a) previous thrombosis and ACA titer > 40 units were independent predictors of subsequent vascular complications; b) a history of miscarriage or thrombosis is significantly associated with adverse pregnancy outcome; c) hematological malignancies can develop during follow-up and patients with APA should be considered at increased risk of developing NHL. Thus the possibility of a hematologic neoplastic disease should be borne in mind in the initial evaluation and during the follow-up of these patients.
The latest initiative of the Registry was the proposal of an international, randomized clinical trial (WAPS study) aimed at assessing the efficacy and safety of high-dose warfarin in preventing recurrent thrombosis in patients with APA and vascular disease. The study is scheduled to start in March 1997.
抗磷脂抗体（APA）的临床重要性源于其与静脉和动脉血栓形成、反复胎儿丢失及血小板减少综合征（称为抗磷脂综合征，APS）的关联。意大利抗磷脂抗体登记处成立于1989年，旨在收集大量狼疮抗凝物（LA）或抗心磷脂抗体（ACA）患者进行临床研究，以便获取更多关于APS临床特征的信息。
意大利登记处已完成两项临床研究，并提议开展一项关于APS患者治疗的国际试验。本文对登记处的这些活动进行了综述。还从《科学引文索引》和《医学索引》收录期刊上发表的相关文章和摘要中获取了更多信息。
登记处的第一项研究是对入组患者的回顾性分析，结果显示：a）特发性APA或继发于系统性红斑狼疮的APA患者中，血栓形成和血小板减少的患病率相似；b）严重血小板减少患者的血栓形成率显著降低，但血小板计数仅轻度降低的患者中未出现这种情况。第二项研究是对该疾病自然史的前瞻性调查，结果显示：a）既往血栓形成和ACA滴度>40单位是后续血管并发症的独立预测因素；b）流产或血栓形成史与不良妊娠结局显著相关；c）随访期间可发生血液系统恶性肿瘤，APA患者发生非霍奇金淋巴瘤（NHL）的风险增加。因此，在对这些患者进行初始评估和随访时应考虑血液系统肿瘤性疾病的可能性。
登记处的最新举措是提议开展一项国际随机临床试验（WAPS研究），旨在评估大剂量华法林预防APA和血管疾病患者复发性血栓形成的疗效和安全性。该研究计划于1997年3月开始。
