Extra-criteria manifestations such as thrombocytopenia and livedo are described associated with antiphospholipid syndrome (APS) but are not included in the current classification criteria. Their clinical expression might be important, as they may be associated with a high-risk profile of antiphospholipid antibodies (aPL) and thrombosis. We evaluated the association between the presence of extra-criteria manifestations in primary obstetric-APS (POAPS) and aPL profiles. We also evaluated whether the presence of extra-criteria manifestations in POAPS patients increases the risk of developing thrombosis during the follow-up period (median follow-up 5 years; range 3-9 years). We selected 79 women who were included in our study only if they were first diagnosed with POAPS (with no history of previous thrombosis) and reevaluated for the presence of thrombosis after the follow-up period. We evaluated the association between the aPL profile and extra-criteria manifestations. We also evaluated the relationship of thrombosis during the follow-up period with extra-criteria manifestations and other risk factors. Patients with three or more extra-criteria manifestations presented high rates of triple positivity for the aPL profile (75%) (p < 0.001). We also found a relationship between the presence of extra-criteria manifestations and the presence of high titers of aPL: 91.7% of patients with three or more extra-criteria manifestations had high titers of aPL (p < 0.01). We further evaluated the group of POAPS patients according to thrombotic events during the follow-up. Among these patients, 6 (7.6%) presented thrombosis. Notably, 100% of patients with a thrombotic event during the follow-up had more than three extra-criteria manifestations. POAPS patients with extra-criteria manifestations might have a high-risk aPL profile and a major risk of developing thrombosis.

中文翻译：

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抗磷脂抗体和血栓形成的高风险实验室概况与产科抗磷脂综合征中的大量额外标准表现有关。

描述了与血小板减少症和活体等超标准症状相关的抗磷脂综合症（APS），但目前的分类标准中并未包括。它们的临床表达可能很重要，因为它们可能与抗磷脂抗体（aPL）和血栓形成的高风险有关。我们评估了原发性产科APS（POAPS）和aPL配置文件中存在的超标准表现之间的关联。我们还评估了在随访期间（中位随访期为5年；范围为3-9年），POAPS患者中是否存在超标准表现会增加发生血栓形成的风险。我们选择了79名仅在首次诊断为POAPS（无先前血栓形成史）并在随访期后重新评估是否存在血栓形成的情况下纳入研究的女性。我们评估了aPL配置文件和标准以外的表现之间的关联。我们还评估了随访期间血栓形成与超标准表现和其他危险因素的关系。具有三个或更多超标准表现的患者对aPL谱表现出较高的三重阳性率（75％）（p <0.001）。我们还发现超标准表现的存在与高滴度aPL之间的关系：91.7％的三个或更多超临界表现的患者具有高滴定度aPL（p <0.01）。我们根据随访期间的血栓事件进一步评估了POAPS患者组。在这些患者中，有6名（7.6％）出现血栓形成。值得注意的是，在随访期间发生血栓事件的患者中，有100％的患者具有超过三种的超标准表现。具有超标准表现的POAPS患者可能具有高风险的aPL状况和发生血栓形成的主要风险。