Li S, Lan X L, Cai H C, Zhu T N, Wang S J, Zhao Y Q
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2021 Jan 14;42(1):33-38. doi: 10.3760/cma.j.issn.0253-2727.2021.01.007.
To cross-sectionally analyze the clinical characteristics of primary antiphospholipid syndrome (PAPS) patients with thrombocytopenia, risk factors associated with thrombocytopenia, and risk of symptom recurrence in these patients. The inpatients with PAPS were retrospectively analyzed in Peking Union Medical College Hospital from 2009 to 2019. Using the collected clinical and laboratory data, the clinical characteristics and risk of symptom recurrence in the PAPS patients with thrombocytopenia were compared with those in the PAPS patients with normal platelet counts. Univariate and multivariate logistic regression analyses were performed to screen the risk factors for thrombocytopenia. In this study, 127 patients with PAPS were enrolled, of which 36 (28.3% ) had thrombocytopenia, with a median age of 38 years, and 63.9% were female. In the thrombocytopenia group, the average platelet count was (58.9±27.0) ×10(9)/L, and the prevalence of thrombosis and morbid pregnancy was not significantly different from that in the normal platelet group. However, the thrombocytopenia group had higher incidence rate of autoimmune hemolytic anemia (19.4% 3.3% ) , livedo reticularis (16.7% 3.3% ) , chronic kidney disease (25% 8.8% ) and antiphospholipid antibodies triple positiveness (61.1% 37.4% ) , lower complement levels (C3 of 0.87 g/L 1.07 g/L, C4 of 0.12 g/L 0.18 g/L, <0.05) , and higher adjusted Global APS Score (median score of 13 9, =0.037) than the normal platelet group. In multivariate logistic regression analysis, hypocomplementemia ( value 5.032, 95% 3.118-22.095) is an independent risk factor for thrombocytopenia. In patients with PAPS, thrombocytopenia is mostly mild to moderate. Hypocomplementemia may be the independent risk factor for thrombocytopenia in PAPS patients. The PAPS patients with thrombocytopenia may have a higher risk of symptom recurrence.
横断面分析原发性抗磷脂综合征(PAPS)血小板减少患者的临床特征、血小板减少相关危险因素及这些患者症状复发风险。对2009年至2019年北京协和医院住院的PAPS患者进行回顾性分析。利用收集的临床和实验室数据,比较血小板减少的PAPS患者与血小板计数正常的PAPS患者的临床特征和症状复发风险。进行单因素和多因素逻辑回归分析以筛选血小板减少的危险因素。本研究共纳入127例PAPS患者,其中36例(28.3%)有血小板减少,中位年龄38岁,63.9%为女性。血小板减少组平均血小板计数为(58.9±27.0)×10⁹/L,血栓形成和病态妊娠的患病率与血小板正常组无显著差异。然而,血小板减少组自身免疫性溶血性贫血(19.4%对3.3%)、网状青斑(16.7%对3.3%)、慢性肾脏病(25%对8.8%)和抗磷脂抗体三联阳性(61.1%对37.4%)的发生率更高,补体水平更低(C3为0.87g/L对1.07g/L,C4为0.12g/L对0.18g/L,P<0.05),且校正后的全球APS评分更高(中位评分为13对9,P=0.037)。多因素逻辑回归分析显示,低补体血症(比值比5.032,95%可信区间3.118 - 22.095)是血小板减少的独立危险因素。在PAPS患者中,血小板减少大多为轻至中度。低补体血症可能是PAPS患者血小板减少的独立危险因素。血小板减少的PAPS患者可能有更高的症状复发风险。