Abstract:
Essential thrombocythemia (ET) is associated with an increased risk of thrombosis and autoimmune renal involvement. We report an extremely rare case of an acute kidney injury (AKI) in the presence of bilateral renal pelvises fibrosis in a patient with a proven diagnosis of ET. A 48-year-old male patient with a past medical history of mild chronic kidney disease and ET was admitted to our hospital with AKI. The patient discontinued his hydroxyurea treatment for the past 2 months and laboratory data showed increasing serum creatinine levels and platelet counts with increased renal sizes, severe hydrocalyx, and bilateral renal sinuses\' fibrosis in imaging. The patient started again on hydroxyurea therapy and showed improvement in all laboratory scales. ET and increased levels of platelet-derived growth factors could cause renal sinuses fibrosis and glomerulopathy. In ET patients with renal sinuses\' fibrosis and glomerulopathy, initiating cytoreductive therapy could improve the outcome.
摘要:
原发性血小板增多症(ET)与血栓形成和自身免疫性肾脏受累的风险增加有关。我们报告了在确诊为ET的患者中,在存在双侧肾盂纤维化的情况下发生急性肾损伤(AKI)的极为罕见的病例。一名48岁男性患者,既往有轻度慢性肾病和ET病史,因AKI入院。患者在过去2个月中停止了羟基脲治疗,实验室数据显示血清肌酐水平和血小板计数随着肾脏大小的增加而增加。严重的水萼,和双侧肾窦影像学纤维化。患者再次开始接受羟基脲治疗,并在所有实验室规模中均显示出改善。ET和血小板源性生长因子水平升高可引起肾窦纤维化和肾小球病。在有肾窦纤维化和肾小球病的ET患者中,启动细胞减灭性治疗可以改善预后.