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Evans 综合征合并急性肾损伤。

Evans Syndrome with Acute Kidney Injury.

机构信息

Department of Nephrology Medicine, The first Affiliated Hospital of Shantou University Medical College, Shantou, China.

出版信息

Arch Iran Med. 2019 Jun 1;22(6):336-339.

Abstract

Evans syndrome is a rare syndrome associated with the simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and idiopathic thrombocytopenic purpura (ITP). Furthermore, acute kidney injury (AKI) is a syndrome characterized by the rapid loss of kidney excretory function and is most often secondary to extrarenal events. However, AKI has rarely been recorded in Evans syndrome without systemic autoimmune disease and malignant tumors of the blood and lymphatic system. Herein, we report the case of a patient who exhibited Evans syndrome presenting with AKI. A 73-year-old woman presented with diarrhea, anuria, low platelet count, and developed a progressive increase of blood urea nitrogen and serum creatinine, as well as anemia with a positive direct Coombs test. We excluded hemolytic uremic syndrome, ITP, and leukemia. Treatment with antibiotics, rehydration therapy, and hemodialysis resulted in partial remission; thus, we diagnosed the patient with Evans syndrome presenting with AKI. The patient was successfully treated by the addition of steroid treatment. When AKI presents with hemolysis and thrombocytopenia, physicians should consider Evans syndrome, which can be appropriately treated when detected early.

摘要

Evans 综合征是一种罕见的综合征,与自身免疫性溶血性贫血 (AIHA) 和特发性血小板减少性紫癜 (ITP) 的同时或序贯发展有关。此外,急性肾损伤 (AKI) 是一种以肾脏排泄功能迅速丧失为特征的综合征,最常继发于肾外事件。然而,在没有系统性自身免疫性疾病和血液及淋巴系统恶性肿瘤的情况下, Evans 综合征合并 AKI 很少被记录。在此,我们报告一例表现为 AKI 的 Evans 综合征患者。一名 73 岁女性因腹泻、无尿、血小板计数降低,并出现血尿素氮和血清肌酐逐渐升高、贫血伴直接 Coombs 试验阳性而就诊。我们排除了溶血尿毒综合征、ITP 和白血病。抗生素治疗、补液治疗和血液透析治疗后部分缓解;因此,我们诊断该患者为 Evans 综合征合并 AKI。加用类固醇治疗后患者成功治疗。当 AKI 出现溶血和血小板减少时,医生应考虑 Evans 综合征,如果早期发现可以进行适当治疗。

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