Objectives To investigate the severe haematological involvement in children with SLE and assess its clinical associations, treatments, outcome and damage accrual. Methods The medical charts of children with SLE in whom haematological involvement was observed were reviewed. Severe haematological indices were defined as autoimmune haemolytic anaemia with a haemoglobin concentration < 8 g/dL, thrombocyte count < 30 000/µL, and neutrophil count < 500/µL. Results Among the 224 patients included, 102 (45.5%) displayed severe indices, predominantly at the initial involvement, and most frequently as severe anaemia in 54 (24.1%) and severe thrombocytopenia in 45 (20.1%). Disease activity did not differ according to the presence of severe disease indices. In addition, the presence of severe indices at initial involvement did not affect the damage accrual. However, a higher rate of damage (51.1% vs. 29.9%, p = 0.002) and steroid-induced damage (28.9% vs. 8.2%, p < 0.001) was evident in patients with flares of the haematological system. Regression analysis revealed that rituximab treatment during the initial episode (OR:4.5, p = 0.006) and the presence of anticardiolipin antibodies (OR:2.3, p = 0.014) significantly increases the odds for haematological system flare. However, severe indices at initial involvement did not increase the odds of a haematological flare. Conclusion Severe haematological indices at onset are common but not related with disease outcomes. Prevention of flares is important to improve outcomes, and a more rigorous maintenance strategy would benefit most to children who display haematological indices refractory to conventional immunosuppressants and those with anti-cardiolipin antibodies.

中文翻译：

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系统性红斑狼疮儿童的严重血液学受累及其临床关联


目的 调查 SLE 儿童的严重血液学受累情况，并评估其临床关联、治疗、结果和损害。方法回顾了观察到血液系统受累的SLE儿童的病历。严重血液学指标定义为自身免疫性溶血性贫血，血红蛋白浓度< 8 g/dL，血小板计数< 30 000/μL，中性粒细胞计数< 500/μL。结果 在纳入的 224 名患者中，102 名（45.5%）患者表现出严重的症状，主要是在初次受累时，最常见的是 54 名（24.1%）名患者出现严重贫血，45 名（20.1%）名患者出现严重血小板减少。疾病活动度并没有根据严重疾病指数的存在而有所不同。此外，最初受累时存在严重指标并不影响损害的累积。然而，血液系统急性发作患者的损伤率（51.1% vs. 29.9%，p = 0.002）和类固醇引起的损伤（28.9% vs. 8.2%，p < 0.001）明显较高。回归分析显示，初次发作期间的利妥昔单抗治疗（OR：4.5，p = 0.006）和抗心磷脂抗体的存在（OR：2.3，p = 0.014）显着增加了血液系统发作的几率。然而，最初受累时的严重指标并没有增加血液病发作的几率。结论 发病时严重的血液学指标很常见，但与疾病结果无关。预防耀斑对于改善预后非常重要，更严格的维持策略对于血液学指标对传统免疫抑制剂无效的儿童和具有抗心磷脂抗体的儿童最有利。