血小板减少与抗磷脂综合征临床及实验室特点的关联分析

The association between thrombocytopenia and clinical and laboratory characteristics in antiphospho-lipid syndrome

目的探讨以血小板减少为主要表现的APS患者的临床及实验室特点,以及将血小板减少纳入分类标准对APS诊断率的影响。方法回顾性分析2003年1月至2017年8月于北京大学人民医院住院的临床资料齐全的APS患者161例,根据血小板减少病史,分为血小板减少组和血小板正常组,采用χ^(2)检验或Fisher确切概率法及t检验进行统计学处理。结果161例APS患者中,原发性APS 48例(29.8%),继发性APS 113例(70.2%)。91例(56.5%)患者出现血小板减少,62例(38.5%)患者以血小板减少首发。原发性APS患者血小板减少组发生血栓(40.0%和69.6%,χ^(2)=4.218,P=0.040)的比例相对较低,静脉血栓(20.0%和47.8%,χ^(2)=4.174,P=0.041)的发生率明显低于血小板正常组;继发性APS患者血小板减少组发生血栓(43.9%和80.9%,χ^(2)=15.496,P<0.01)的比例相对较低,其中动脉血栓(21.2%和42.6%,χ^(2)=5.944,P=0.015)及静脉血栓(22.7%和51.1%,χ^(2)=9.753,P=0.002)的发生率均明显低于血小板正常组。在实验室检查方面,原发性APS患者血小板减少组较易出现抗心磷脂抗体(aCL)阳性(80.0%和52.2%,χ^(2)=4.174,P=0.041),继发性APS患者血小板减少组较易出现白细胞减少(25.8%和10.6%,χ^(2)=4.002,P=0.045)、补体C3下降(78.7%和44.4%,χ^(2)=13.205,P<0.01)和补体C4下降(74.6%和46.7%,χ^(2)=8.485,P=0.004)。在本组患者中,符合1988年、1989年、1999年及2006年APS的分类标准的患者比例分别为88.2%、87.0%、64.0%及70.8%,若将血小板减少纳入到2006年APS分类标准中,将使该比例提高到96.3%。结论伴有血小板减少的APS患者发生血栓的风险显著低于血小板正常者。血小板减少是APS的重要表现,可提高APS的临床诊断率。

Objective To analyze the association between thrombocytopenia and clinical manifestations in patients with antiphospholipid syndrome(APS),and the significance of adding thrombocytopenia to the classification criteria of APS.Methods One hundred and sixty one APS patients admitted to Peking University People's Hospital from January 2003 to August 2017 were retrospectively analyzed.Clinical and laboratory data were compared between patients with and without thrombocytopenia.Chi-square test,Fisher's exact test and t test were used for statistical analysis.Results Among 161 APS patients,48 patients(29.8%)were primary APS and 113 patients(70.2%)were secondary APS.Ninety-one patients(56.5%)experienced thrombocytopenia,while 62 patients(38.5%)had thrombocytopenia as the initial symptom.Among primary APS patients,compared with patients without thrombocytopenia,the prevalence of thrombosis was significantly lower in patients with thrombocytopenia(40.0%vs 69.6%,χ^(2)=4.218,P=0.040),especially venous thrombosis(20.0%vs 47.8%,χ^(2)=4.174,P=0.041).Among secondary APS patients,compared with patients without thrombocyt-openia,the prevalence of thrombosis,arterial thrombosis(21.2%vs 42.6%,χ^(2)=5.944,P=0.015)and venous thrombosis(22.7%vs 51.1%,χ^(2)=9.753,P=0.002)was significantly lower in patients with thrombocytopenia(43.9%vs 80.9%,χ^(2)=15.496,P<0.01).As for laboratory findings,primary APS patients with thrombocytopenia showed a higher proportion of positive aCL findings(80.0%vs 52.2%,χ^(2)=4.174,P=0.041),while secondary APS patients with thrombocytopenia experienced more leukopenia(25.8%vs 10.6%,χ^(2)=4.002,P=0.045),lower complement C3 levels(78.7%vs 44.4%,χ^(2)=13.205,P<0.01)and complement C4 levels(74.6%vs 46.7%,χ^(2)=8.485,P=0.004).The proportion of patients fulfilling 1988,1989,1999 and 2006 APS classification criteria in our cohort was 88.2%,87.0%,64.0%and 70.8%,respectively.Inclusion of thrombocytopenia into 2006 criteria improved the proportion to 96.3%.Conclusion The prevalence of thrombosis in APS patients with thrombocytopenia is significantly low.Thrombocytopenia is an important clinical manifestation of APS which can improve the diagnostic accuracy of APS.