Thakur Neha, Chandra Jagdish, Dhingra Bhavna, Singh V
Department of Pediatrics, Kalawati Saran Children Hospital, New Delhi, 110001 India.
Indian J Hematol Blood Transfus. 2015 Mar;31(1):68-70. doi: 10.1007/s12288-014-0357-5. Epub 2014 Mar 14.
Systemic lupus erythematosus (SLE) is a chronic, multisystem, autoimmune disease characterized by periods of increased disease activity caused by inflammation of blood vessels and connective tissue. Pediatric patients with SLE have a more severe clinical course when compared with adults. Patients commonly present with rash, fever, and arthritis, although the presentation may be unpredictable. Hematological findings are more predominant in children than adults. Thirty-nine percent of children with SLE will develop hematological abnormalities, one of the American Rheumatic Association criteria for classifying the disease. In our case series we found varied hematological picture and presentation. We present here four case reports of SLE cases with interesting hematological features. Our first case is a 13 month old female child who was initially diagnosed as Evans syndrome and 2 years later diagnosed as SLE. Second case is a 3 year old male child who had SLE with warm antibody AIHA. Third case is a 6 year old female child who presented with AIHA and was diagnosed with SLE 6 years later. Fourth case is a 6 year old female child diagnosed as SLE with aplastic anemia. Hematological findings should be carefully assessed and treated in order to decrease disease related morbidity.
系统性红斑狼疮(SLE)是一种慢性、多系统自身免疫性疾病,其特征是血管和结缔组织炎症导致疾病活动期增加。与成人相比,儿童SLE患者的临床病程更为严重。患者通常表现为皮疹、发热和关节炎,尽管表现可能不可预测。血液学表现在儿童中比成人更为突出。39%的SLE儿童会出现血液学异常,这是美国风湿病学会对该疾病进行分类的标准之一。在我们的病例系列中,我们发现了不同的血液学表现。我们在此展示四例具有有趣血液学特征的SLE病例报告。我们的第一例是一名13个月大的女童,最初被诊断为伊文氏综合征,2年后被诊断为SLE。第二例是一名3岁男童,患有SLE伴温抗体自身免疫性溶血性贫血(AIHA)。第三例是一名6岁女童,出现AIHA,6年后被诊断为SLE。第四例是一名6岁女童,被诊断为SLE伴再生障碍性贫血。应仔细评估和治疗血液学表现,以降低疾病相关的发病率。