系统性红斑狼疮伴持续性血小板计数增多的临床分析

背景　系统性红斑狼疮（SLE）伴持续性血小板计数增多不常见，其原因及机制尚不明确，相关研究较少，且国内未见相关报道。目的　探讨SLE伴持续性血小板计数增多的临床特点及原因。方法　回顾性分析2010-2019年在淄博市第一医院诊断的393例SLE患者，其中4例出现持续血小板计数增多作为研究组，随机选取同期在本院就诊的25例未出现持续血小板计数增多的SLE患者作为对照组。收集SLE合并血小板计数增多患者的性别、年龄、血小板计数增多与确诊SLE时间间隔、血小板计数增多持续时间、器官受累数、血常规、红细胞沉降率（ESR）、C反应蛋白（CRP）、补体、24 h尿蛋白定量、抗核抗体（ANA）、抗双链DNA抗体（抗dsDNA抗体）、抗心磷脂抗体（ACA）、狼疮抗凝物（LA）、骨髓形态学检查、JAK2/V617F基因突变、系统性红斑狼疮疾病活动度指数（SLE-DAI）评分及脾显像结果等。结果　4例SLE伴持续血小板计数增多，患者均为女性，平均年龄（36.3±15.1）岁，血小板计数为732×109/L~1 439×109/L，中位数为903×109/L；血小板计数增多与确诊SLE时间间隔中位数为3年（2~7年），血小板计数增多持续时间中位数为12个月（8~28个月）；1例ESR升高，1例CRP升高，3例补体C3降低，2例补体C4降低，2例抗dsDNA抗体阳性，3例ACA阳性、2例LA阳性，SLE-DAI评分中位数为5分（2~10分），血小板计数增多原因1例为合并原发血小板增多症（ET），2例为脾功能减退，1例原因不明。研究组血小板计数、ACA阳性率、LA阳性率高于对照组（P<0.05）。结论　SLE伴持续性血小板计数增多不常见，脾功能减退是主要原因，合并ET也是一项重要原因，血小板计数增多可能与ACA、LA有相关性。

Clinical Analysis of Systemic Lupus Erythematosus with Persistent Thrombocytosis

Background　It is uncommon for systemic lupus erythematosus（SLE） with persistent thrombocytosis，whose causes and pathogenesis remain to be unclear.There are few related studies，and no related reports in China in particular.Objective　To investigate the clinical features and etiology of SLE with persistent thrombocytosis.Methods　The medical records of 393 cases diagnosed with SLE in the First Hospital of Zibo from 2010 to 2019 were retrospectively analyzed.Among them，4 with persistent thrombocytosis and 25 randomly sampled cases without persistent thrombocytosis were assigned to the study group，and control group，respectively.Gender，age，the interval between thrombocytosis and timing of diagnosing SLE，duration of thrombocytosis，number of organs involved，routine blood parameters，erythrocyte sedimentation rate（ESR），C-reactive protein（CRP），complement，24-hour urine protein，antinuclear antibody（ANA），anti-dsDNA antibody，anti-cardiolipin antibody（ACA），lupus anticoagulant（LA），bone marrow morphology，JAK2/v617F mutation，SLEDAI score，selective spleen scintigraphy and others were collected.Results　The 4 cases with persistent thrombocytosis were female，with a mean age of （36.3±15.1） years，median platelet count of 903×109/L（range，732×109/L-1 439×109/L），median interval between thrombocytosis and timing of diagnosing SLE of 3（range，2-7） years，median duration of thrombocytosis of 12（range，8-28） months，and median SLEDAI score of 5（range，2-10）.1 with ESR elevated，1 with CRP elevated，3 with C3 hypocomplementemia，2 with C4 hypocomplementemia，2 with positive anti-dsDNA antibody，3 with positive ACA and 2 with positive LA.In terms of the causes of thrombocytosis：1 was ascribed to essential thrombocythemia，2 were ascribed to hyposplenism，and 1 was cryptogenic.The levels of platelet count，ACA and LA in study group were higher than those of control group（P<0.05）.Conclusion　SLE with persistent thrombocytosis is rarely reported，with hyposplenism as one possible major cause，and essential thrombocythemia in SLE another one.Thrombocytosis was associated with ACA and LA.