Hacettepe University, School of Medicine, Department of Pediatric Hematology, Ankara, Turkey.
Lupus. 2012 Jul;21(8):878-84. doi: 10.1177/0961203312443721. Epub 2012 Apr 11.
The aim of this study was to analyze the hematological features in children with systemic lupus erythematosus (SLE) and to review our current treatment protocols.
We evaluated hematological findings of 43 children with SLE diagnosed and followed at the Pediatric Rheumatology Division of Hacettepe University, Turkey. Thirty-seven patients with hematological abnormalities were analyzed in detail.
Median age at presentation was 13 years. Hematological involvement was seen in 86% of patients. The most common hematological finding was anemia (n = 30). Anemia was either a Coombs (+) hemolytic one, or was due to other causes. Hemolytic anemia was treated with steroids and intravenous gamma globulin (IVIG). Leucopenia and thrombocytopenia were detected in 35.1 % and 37.8 %, respectively. Bone marrow aspiration was performed in 15, mainly for cytopenia. Secondary dysplastic changes were common. Acute lymphoblastic leukemia (ALL) was diagnosed in one patient. Six patients were diagnosed as having macrophage activation syndrome (MAS). One patient died due to secondary infections and multiorgan failure despite aggressive treatment. In patients diagnosed early, treatment with steroids and cyclosporine resulted in an excellent response. Thrombotic microangiopathy was detected in two patients. Both were treated successfully with steroids and plasma exchange. Antiphospholipid and anticardiolipin antibodies were positive in 12 and 15 of the patients, respectively. Five developed deep vein thrombosis (DVT), one cerebral sinus thrombosis and one presented with purpura fulminans. They were effectively treated with anticoagulation protocol.
Hematological findings should be carefully assessed and treated vigorously to prevent the morbidity and possible mortality.
本研究旨在分析系统性红斑狼疮（SLE）患儿的血液学特征，并回顾我们目前的治疗方案。
我们评估了在土耳其哈塞特佩大学儿科风湿病科诊断和随访的 43 例系统性红斑狼疮患儿的血液学发现。详细分析了 37 例有血液学异常的患者。
中位发病年龄为 13 岁。86%的患儿存在血液学受累。最常见的血液学发现是贫血（n=30）。贫血要么是 Coombs（+）溶血性贫血，要么是由其他原因引起的。溶血性贫血用类固醇和静脉注射免疫球蛋白（IVIG）治疗。白细胞减少和血小板减少分别为 35.1%和 37.8%。为了治疗血细胞减少症，15 名患儿进行了骨髓抽吸，主要是为了治疗血细胞减少症。继发性发育不良改变很常见。一名患儿被诊断为急性淋巴细胞白血病（ALL）。6 名患儿被诊断为巨噬细胞活化综合征（MAS）。一名患儿尽管进行了积极治疗，但由于继发感染和多器官衰竭而死亡。在早期诊断的患者中，类固醇和环孢素治疗可获得良好的疗效。两名患者被诊断为血栓性微血管病。两者均经类固醇和血浆置换成功治疗。12 例患者抗磷脂和抗心磷脂抗体阳性，15 例患者抗心磷脂抗体阳性。5 例患者发生深静脉血栓形成（DVT），1 例患者发生脑窦血栓形成，1 例患者发生暴发性紫癜。他们通过抗凝方案得到了有效治疗。
应仔细评估血液学发现并积极治疗，以预防发病率和可能的死亡率。
