Galli M, Finazzi G, Barbui T
a Division of Haematology , Ospedali Riuniti , Bergamo , Italy.
Hematology. 1996;1(2):125-32. doi: 10.1080/10245332.1996.11746296.
The Antiphospholipid Syndrome (APS) is defined by the association between antiphospholipid antibodies, i.e. anticardiolipin (aCL) and/or lupus anticoagulant (LA) antibodies, and one or more of the following clinical manifestations: arterial and venous thrombosis, recurrent abortions and thrombocytopenia (1). Among them, deep venous thromboses, pulmonary embolism and thrombosis of the cerebral arteries are the most frequent events, occurring in approximately 1/3 of the patients. Thrombosis of the placental vessels (2) is considered the cause of the obstetrical complications (recurrent spontaneous abortions, fetal deaths or fetal growth retardation) suffered by approximately 10% of the women with antiphospholipid antibodies (1), whereas a variable degree of thrombocytopenia is reported by about 20-25% of the patients (3). Less commonly, skin necrosis, livedo reticularis, hemolytic anemia, dementia or other neuropsychiatric events and the so-called "catastrophic" APS may also develop in the setting of APS (4, 5). Two types of APS have been described: the "Primary" APS, which occurs in the absence of an underlying disease (6), and the "Secondary" APS, which is related to Systemic Lupus Erythematosus (SLE), other autoimmune or neoplastic diseases or other pathological conditions (7). Noteworthy, a substantial amount of patients suffering from APS are young: 50% of the patients enrolled in the Italian Registry of Antiphospholipid Antibodies were aged less than 40 years (8).
抗磷脂综合征(APS)的定义是抗磷脂抗体,即抗心磷脂(aCL)和/或狼疮抗凝物(LA)抗体,与以下一种或多种临床表现相关:动脉和静脉血栓形成、反复流产和血小板减少症(1)。其中,深静脉血栓形成、肺栓塞和脑动脉血栓形成是最常见的事件,约三分之一的患者会出现。胎盘血管血栓形成(2)被认为是约10%抗磷脂抗体女性患者发生产科并发症(反复自然流产、胎儿死亡或胎儿生长受限)的原因(1),而约20-25%的患者报告有不同程度的血小板减少症(3)。较少见的情况下,皮肤坏死、网状青斑、溶血性贫血、痴呆或其他神经精神事件以及所谓的“灾难性”APS也可能在APS患者中发生(4,5)。已描述了两种类型的APS:“原发性”APS,发生在无基础疾病的情况下(6),以及“继发性”APS,与系统性红斑狼疮(SLE)、其他自身免疫性或肿瘤性疾病或其他病理状况相关(7)。值得注意的是,大量患有APS的患者较为年轻:意大利抗磷脂抗体登记处登记的患者中有50%年龄小于40岁(8)。