合并嗜酸性粒细胞增多,出现Purtscher样视网膜病变的非典型溶血性尿毒症一例

A Case of aHUS with Eosinophilia and Purtscher-like Retinopathy

  • 摘要: 非典型溶血性尿毒症(atypical hemolytic uremic syndrome,aHUS)是一种由补体异常所引发的罕见病,临床表现以微血管病性溶血性贫血、血小板减少症和急性肾损伤为主要特征。本文报道1例以严重肾功能不全伴双眼视力急进性下降起病的患者,病程中出现嗜酸性粒细胞增多症,排除其他疾病后诊断为aHUS。予以激素治疗后嗜酸性粒细胞降至正常,血浆置换联合依库珠单抗治疗后肾脏功能趋于稳定,血小板恢复正常,但视力无明显改善。本文回顾该患者的诊治历程并结合文献复习,以期为临床医师提供借鉴。

     

    Abstract: A typical hemolytic uremic syndrome (aHUS), a rare disease caused by complement abnormalities, is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. In this paper, we report a patient with severe renal insufficiency with rapidly progressive decline in binocular visual acuity, who developed eosinophilia during the course of the disease, and was diagnosed with aHUS after excluding other diseases. After glucocorticoid treatment, eosinophils decreased to normal, and after treatment with plasmapheresis combined with eculizumab, renal function tended to be stable, platelets returned to normal, but visual acuity did not improve significantly. This article reviews the diagnosis and treatment process of this patient and incorporates the review of literature, in the hope of providing reference for clinicians.

     

/

返回文章
返回