Dadkhah Adeleh, Bagheri Seyed Morteza, Rakhshankhah Nima
Department of Diagnostic Radiology, Iran University of Medical Sciences, Hemat Highway next to Milad Tower, Tehran, 14535, Iran.
Department of Radiology, Hasheminejad Kidney Center (HKC), Iran University of Medical Sciences, Tehran, Iran.
Radiol Case Rep. 2023 Jul 25;18(10):3471-3474. doi: 10.1016/j.radcr.2023.07.016. eCollection 2023 Oct.
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.
原发性血小板增多症(ET)与血栓形成风险增加及自身免疫性肾脏受累有关。我们报告了一例极为罕见的病例,一名确诊为ET的患者出现双侧肾盂纤维化并伴有急性肾损伤(AKI)。一名48岁男性患者,既往有轻度慢性肾脏病和ET病史,因AKI入院。该患者在过去2个月停用了羟基脲治疗,实验室数据显示血清肌酐水平升高、血小板计数增加,影像学检查显示肾脏大小增大、严重肾盏积水以及双侧肾窦纤维化。患者再次开始羟基脲治疗后,所有实验室指标均有改善。ET和血小板衍生生长因子水平升高可导致肾窦纤维化和肾小球病变。对于有肾窦纤维化和肾小球病变的ET患者,启动细胞减灭治疗可改善预后。