Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan.
Division of Hemodialysis and Apheresis, Okayama University Hospital, Japan.
Intern Med. 2021 Apr 1;60(7):1055-1060. doi: 10.2169/internalmedicine.5976-20. Epub 2020 Oct 28.
A 65-year-old woman with systemic sclerosis and systemic lupus erythematosus developed acute kidney injury (AKI), Coombs-positive autoimmune hemolytic anemia and autoimmune thrombocytopenia; therefore, she was diagnosed with Evans syndrome (ES). Intravascular hemolysis was suggested as the cause of AKI based on the presence of acute tubular injury and trace hemosiderin deposits on the renal biopsy. The renal function, hemolytic anemia and thrombocytopenia were restored by an increased dose of glucocorticoids, hemodialysis, and plasma exchange. Although ES with severe hemolytic anemia is very rare, it is important to detect possible renal dysfunction when encountering patients with severe hemolysis.
一位 65 岁女性，患有系统性硬化症和系统性红斑狼疮，出现急性肾损伤（AKI）、抗人球蛋白试验阳性自身免疫性溶血性贫血和自身免疫性血小板减少症；因此，她被诊断为 Evans 综合征（ES）。基于肾活检中存在急性肾小管损伤和微量含铁血黄素沉积，提示血管内溶血是 AKI 的病因。通过增加糖皮质激素剂量、血液透析和血浆置换，肾功能、溶血性贫血和血小板减少得到恢复。尽管严重溶血性贫血的 ES 非常罕见，但在遇到严重溶血的患者时，检测可能的肾功能障碍非常重要。
