Mudarres Mohamed Fawzi, Azrieh Bahjat
Nephrology, University of Iowa Hospitals and Clinics, Iowa, USA.
Cureus. 2024 Sep 6;16(9):e68809. doi: 10.7759/cureus.68809. eCollection 2024 Sep.
Polycythemia vera (PV) is a rare myeloproliferative neoplasm characterized by the clonal proliferation of hematopoietic stem cells, leading to an elevated red blood cell mass. This hyperproliferative state increases blood viscosity and predisposes patients to thrombotic events, which are a significant cause of morbidity and mortality in PV. The diagnosis of PV is typically confirmed through elevated hemoglobin or hematocrit levels, low serum erythropoietin, and the presence of the Janus kinase 2 (JAK2) mutation. Common complications include venous and arterial thromboses, hemorrhage, and transformation to myelofibrosis or acute leukemia. A 68-year-old female with a history of PV and chronic kidney disease (CKD) presented with uremic symptoms in the form of malaise and nausea. Laboratory investigations indicated acute kidney injury (AKI) and hyperkalemia. Imaging evaluation of renal US Doppler revealed renal artery thrombosis and an incidental adrenal hemorrhage. The patient was managed with intravenous heparin and did not receive thrombolytics or thrombectomy. Her renal function did not improve, necessitating the initiation of hemodialysis (HD) during hospitalization. Over the course of the next few weeks, her renal parameters improved and she managed to be discharged from dialysis. The primary goal of this study was to highlight a rare presentation of renal artery thrombosis secondary to polycythemia vera (PV) and discuss the complexities involved in managing the underlying disease and its thrombotic complication, particularly in the presence of concomitant bleeding. Effective management of PV-related thrombosis requires a delicate balance between anticoagulation to prevent further thrombotic events while carefully addressing the risk of hemorrhage.
真性红细胞增多症(PV)是一种罕见的骨髓增殖性肿瘤,其特征是造血干细胞的克隆性增殖,导致红细胞量增加。这种增殖状态会增加血液粘度,使患者易发生血栓事件,而血栓事件是PV患者发病和死亡的重要原因。PV的诊断通常通过血红蛋白或血细胞比容水平升高、血清促红细胞生成素降低以及存在Janus激酶2(JAK2)突变来确诊。常见并发症包括静脉和动脉血栓形成、出血以及转化为骨髓纤维化或急性白血病。一名68岁有PV病史和慢性肾脏病(CKD)的女性,以不适和恶心的形式出现尿毒症症状。实验室检查显示急性肾损伤(AKI)和高钾血症。肾脏超声多普勒成像评估显示肾动脉血栓形成和偶然发现的肾上腺出血。该患者接受了静脉肝素治疗,未接受溶栓或血栓切除术。她的肾功能没有改善,住院期间需要开始血液透析(HD)。在接下来的几周里,她的肾脏参数有所改善,最终成功脱离透析出院。本研究的主要目的是强调真性红细胞增多症(PV)继发肾动脉血栓形成的罕见表现,并讨论管理基础疾病及其血栓并发症所涉及的复杂性,特别是在存在合并出血的情况下。PV相关血栓形成的有效管理需要在抗凝以预防进一步血栓事件和谨慎处理出血风险之间取得微妙平衡。