Abstract: Objective　To analyze the characteristics of hematological involvement in juvenile onset systemic lupus erythematosus（jSLE） and their relationship with immunological indicators. Methods　121 initial diagnosed jSLE patients who were hospitalized between May，2012 to August，2019 were retrospectively analyzed for the clinical and laboratory data. Results　All the 112 cases（92.6%） had hematological abnormalities. Totally 55 cases（45.5%） visited hospitals with hematologic abnormality as initial symptoms. Totally 106 cases had anemia（87.6%），including 69 cases（57%） of mild anemia，35 cases（28.9%） of moderate anemia and 2 cases（1.7%） of severe anemia； 42 cases（34.7%） had thrombocytopenia，including 30 cases（24.8%） of mild thrombocytopenia，10 cases（8.3%） of moderate thrombocytopenia and 2 cases（1.7%） of severe thrombocytopenia；68 cases（56.2%） had leukopenia. Among 112 cases，33 cases（29.5%） had one blood line decreased，54 cases（48.2%） had two blood lines decreased，and 25 cases（22.3%） had all three blood lines decreased. Compared to one or two-blood line decreased patients，three-blood line decreased patients had  significant increased dsDNA positive rate and lower levels of complement C3 and C4（all P＜0.05）. Conclusion　About half of jSLE patients visited hospitals due to the hematological abnormality，and one-fifth have pancytopenia. The severity of hematological damage is related to dsDNA positive rate and decreased levels of complement C3 and C4，suggesting that peripheral blood routine test may be an effective indicator to monitor the disease activity of SLE.

Key words: juvenile, systemic lupus erythematosus, hematological system, dsDNA, complement

摘要： 目的　分析儿童系统性红斑狼疮（jSLE）血液系统受累特征及免疫学指标相关性，以提高对疾病的认识。方法　回顾性分析2012年5月至2019年8月期间浙江大学医学院附属儿童医院风湿免疫过敏科初诊住院诊断为jSLE 121例患儿临床及实验室资料。结果　121例患儿中合并血液学异常112例（92.6%）。以血液学异常为首发症状就诊者55例（45.5%）；轻度贫血69例（57%），中度贫血35例（28.9%），重度贫血2例（1.7%）；白细胞减低68例（56.2%）；血小板轻度减少30例（24.8%），中度减少10例（8.3%），重度减少2例（1.7%）。112例中血一系减少33例（29.5%），两系减少54例（48.2%），三系减少25例（22.3%）。与血一系或两系减少患者比较，血三系减少者抗dsDNA抗体阳性率最高，补体C3、C4水平最低（均P＜0.05）。结论　该组半数jSLE患者以血液系统受累为首发症状就诊，1/5存在三系减少；血液系统受累程度与dsDNA阳性率及补体C3、C4下降水平相关，提示血常规可作为监测SLE疾病活动性的指标。

关键词: 儿童, 系统性红斑狼疮, 血液系统, 抗双链DNA抗体, 补体