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原发性抗磷脂综合征中的血小板减少症:与预后的关系及临床意义。

Thrombocytopenia in primary antiphospholipid syndrome: association with prognosis and clinical implications.

机构信息

Department of Rheumatology, Chinese Academy of Medical Sciences and Peking Union Medical College Hospital.

National Clinical Research Center for Dermatologic and Immunologic Diseases.

出版信息

Rheumatology (Oxford). 2022 Dec 23;62(1):256-263. doi: 10.1093/rheumatology/keac264.

DOI:10.1093/rheumatology/keac264
PMID:35536236
Abstract

OBJECTIVES

Thrombocytopenia, a frequent clinical manifestation in patients with APS, could be an independent predictor of recurrent thrombotic, obstetric and severe extracriteria events.

METHODS

This single-centre prospective study enrolled 218 consecutive patients diagnosed with primary APS between 2010 and 2021. Thrombocytopenia was defined as a platelet count less than 100 × 109/L.

RESULTS

Our cohort included 74 (33.94%) patients with thrombocytopenia and 144 patients with a continuous normal platelet count. Comparison of baseline characteristics indicated that patients with thrombocytopenia had more visceral venous thromboses [10 (13.51%) vs 5(3.47%); P = 0.009] and extracriteria manifestations [mainly haemolytic anaemia; 20 (27.03%) vs 17 (11.81%); P = 0.007]. Hypocomplementemia was more likely among patients with thrombocytopenia [19 (25.68%) vs 16 (11.11%); P = 0.01]. The presence of aCL-IgG/IgM, anti-β2-glycoprotein I and lupus anticoagulant were more frequently detected in patients with thrombocytopenia. In survival analysis, thrombotic, obstetric and severe extracriteria survival rates were significantly worse in patients with thrombocytopenia. In multivariate Cox regression, thrombocytopenia was an independent risk factor for all endpoint events, including thrombotic events [hazard ratio (HR) 2.93 (95% CI 1.31, 6.56), P = 0.009], pregnancy morbidity [HR 8.00 (95% CI 2.43, 26.37), P = 0.0006] and severe extracriteria events [HR 15.27 (95% CI 1.85, 125.98), P = 0.01].

CONCLUSION

Thrombocytopenia could identify primary APS patients at high risk of developing thrombotic events, pregnancy morbidity and severe extracriteria events.

摘要

目的

血小板减少症是 APS 患者常见的临床表现,它可能是复发性血栓形成、产科和严重非标准事件的独立预测因子。

方法

这项单中心前瞻性研究纳入了 2010 年至 2021 年期间确诊的原发性 APS 连续 218 例患者。血小板减少症定义为血小板计数<100×109/L。

结果

我们的队列包括 74 例(33.94%)血小板减少症患者和 144 例血小板计数持续正常的患者。基线特征比较表明,血小板减少症患者更易发生内脏静脉血栓形成[10(13.51%)比 5(3.47%);P=0.009]和非标准表现[主要为溶血性贫血;20(27.03%)比 17(11.81%);P=0.007]。血小板减少症患者补体 C3 缺乏更为常见[19(25.68%)比 16(11.11%);P=0.01]。血小板减少症患者更常检测到 aCL-IgG/IgM、抗-β2-糖蛋白 I 和狼疮抗凝物。生存分析显示,血小板减少症患者的血栓形成、产科和严重非标准生存结局明显更差。多因素 Cox 回归分析显示,血小板减少症是所有终点事件的独立危险因素,包括血栓形成事件[危险比(HR)2.93(95%可信区间 1.316.56),P=0.009]、妊娠并发症[HR 8.00(95%可信区间 2.4326.37),P=0.0006]和严重非标准事件[HR 15.27(95%可信区间 1.85~125.98),P=0.01]。

结论

血小板减少症可识别原发性 APS 患者发生血栓形成事件、妊娠并发症和严重非标准事件的风险较高。

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