Hematological Involvement in Pediatric Systemic Lupus Erythematosus

M. Ayala, X. Norambuena, Alfonso Loosli, A. Quezada
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Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with multisystemic involvement with a variable clinical course. Hematological involvement is frequent and both morbidity and mortality, as well as the increased risk of infection and decreased survival rates have been significantly related with such compromise. The objective of this study was to describe the hematologic alterations in pediatric patients with SLE at the time of diagnosis. Methods. A retrospective descriptive study of patients with SLE controlled in the Immunorheumatology outpatients centre of Exequiel Gonzalez Cortes Children´s Hospital, Santiago, Chile was conducted. Thirty-three clinical files were reviewed, and demographic and clinical data were obtained. Results. Thirty patients met the inclusion criteria, 22 females, mean age 11.6 years (range 5 to 14). The prevalence of hematological compromise was 60% (n = 18). The most frequent hematological alteration was lymphopenia in 14 patients, followed by autoimmune hemolytic anemia (HA) in 11, leukopenia in 10, and thrombocytopenia in 3 patients. The association between different hematological alterations showed lymphopenia with leukopenia in 50% of the patients (n = 9) and HA with lymphopenia in 8 patients. The most frequent extra-hematological manifestations in patients with hematological alterations was skin involvement in 26/30 patients, followed by kidney involvement in 25, articular in 22, and serositis in 17. Conclusions. Hematological involvement in this series of pediatric SLE was frequent and this is included in traditional and current diagnostic criteria. In a group of patients, it can be the first manifestation, and this requires a differential diagnosis and careful monitoring. In other cases, it may be a manifestation of severe SLE that requires vigorous treatment.
小儿系统性红斑狼疮的血液学损害
系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病,涉及多系统,临床病程多变。血液学受累是常见的,发病率和死亡率,以及感染风险的增加和生存率的降低都与这种妥协有显著关系。本研究的目的是描述儿科SLE患者在诊断时的血液学改变。方法。对智利圣地亚哥Exequiel Gonzalez Cortes儿童医院免疫风湿病门诊中心控制的SLE患者进行了回顾性描述性研究。我们回顾了33份临床档案,并获得了人口统计学和临床数据。结果。30例患者符合纳入标准,其中女性22例,平均年龄11.6岁(5 ~ 14岁)。血液学损害的患病率为60% (n = 18)。最常见的血液学改变是淋巴细胞减少(14例),其次是自身免疫性溶血性贫血(HA)(11例),白细胞减少(10例)和血小板减少(3例)。不同血液学改变之间的关联显示50%的患者(n = 9)淋巴细胞减少伴白细胞减少,8例HA伴淋巴细胞减少。血液学改变患者最常见的血液学外表现是26/30患者的皮肤受累,其次是25例肾脏受累,22例关节受累,17例血清炎。结论。血液学累及在这一系列的儿童SLE是常见的,这是包括在传统和当前的诊断标准。在一组患者中,它可能是第一个表现,这需要鉴别诊断和仔细监测。在其他情况下,它可能是严重SLE的表现,需要积极治疗。
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