Department of Pediatrics, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, No. 261, Huansha Road, Hangzhou, Zhejiang Province People's Republic of China.
J Int Med Res. 2022 Aug;50(8):3000605221115390. doi: 10.1177/03000605221115390.
We report the cases of two children who presented with autoimmune hemolytic anemia (AIHA) as an initial presentation of systemic lupus erythematosus (SLE). Both patients had a positive Coombs test, anemia, and an increased number of spherocytes in their blood smear. The patient in Case 1 presented with fever, urticarial erythema, facial paresis, AIHA, and leucopenia. Immunological screening revealed low complement protein levels and positive anti-nuclear antibody, anti-double-stranded DNA, and antiphospholipid antibody results. A further laboratory workup revealed a positive lupus anticoagulant (LA) result and low factor II levels. She was diagnosed with lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) in addition to SLE. The patient in Case 2 presented with fever, butterfly rash, thyroid enlargement, leucopenia, and AIHA. She was diagnosed with SLE with thyroiditis. Both patients were started on combined immunosuppressive therapy, and both patients' clinical symptoms finally resolved. A literature review on childhood SLE showed that AIHA is common in patients with SLE. LAHPS is an uncommonly identified cause of bleeding in patients with SLE, and it must be considered when evaluating children with a positive LA result.
我们报告了两例以自身免疫性溶血性贫血 (AIHA) 为首发表现的系统性红斑狼疮 (SLE) 患儿。两位患者的 Coombs 试验均为阳性，存在贫血和血涂片球形红细胞增多。病例 1 患者表现为发热、荨麻疹性红斑、面瘫、AIHA 和白细胞减少。免疫筛选显示补体蛋白水平降低，抗核抗体、抗双链 DNA 和抗磷脂抗体阳性。进一步实验室检查显示狼疮抗凝物 (LA) 阳性和因子 II 水平降低。除 SLE 外，她还被诊断为狼疮抗凝物低凝血酶原血症综合征 (LAHPS)。病例 2 患者表现为发热、蝶形皮疹、甲状腺肿大、白细胞减少和 AIHA。她被诊断为伴有甲状腺炎的 SLE。两位患者均开始接受联合免疫抑制治疗，最终患者的临床症状均得到缓解。对儿童 SLE 的文献复习表明，AIHA 在 SLE 患者中较为常见。LAHPS 是 SLE 患者出血的一种罕见病因，在评估 LA 阳性结果的儿童时必须考虑到这一点。
