Erythrocytosis or polycythemia is defined as an increase in red blood cell concentration above the age- and sex-specific normal levels. Unlike anemia, which is very common in patients with chronic kidney disease (CKD), erythrocytosis is less frequent but requires specific understanding by health care professionals in order to provide the best care. Erythrocytosis, especially when undiagnosed and untreated, can lead to serious thrombotic events and higher mortality. Classic causes of erythrocytosis associated with CKD include cystic kidney diseases, kidney or other erythropoietin-secreting neoplasms, high-altitude renal syndrome, overdosage of erythropoietin-stimulating agents, androgen therapy, heavy smoking, chronic lung disease, obstructive sleep apnea, IgA nephropathy, post–kidney transplant erythrocytosis, renal artery stenosis, and congenital etiologies. After ruling out the common acquired causes of erythrocytosis and/or in the presence of suggestive parameters, primary erythrocytosis or polycythemia vera (PV) should be considered, and patients should be screened for somatic mutation. The newest entity inducing erythrocytosis is linked to the use of sodium/glucose cotransporter 2 (SGLT2) inhibitors that hypothetically activate hypoxia-inducible factor 2α (HIF-2α) and in some cases unmask PV. This Review focuses on the pathogenesis, renal manifestations and management of PV, the pathophysiology of erythrocytosis induced by SGLT2 inhibitors and the relevance of timely mutation screening in these patients.

中文翻译：

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红细胞增多症和 CKD：综述


红细胞增多症或红细胞增多症被定义为红细胞浓度增加超过年龄和性别特定的正常水平。与慢性肾病 (CKD) 患者中常见的贫血不同，红细胞增多症的发生率较低，但需要医疗保健专业人员的具体了解才能提供最佳护理。红细胞增多症，尤其是未经诊断和治疗的情况下，可导致严重的血栓事件和更高的死亡率。与 CKD 相关的红细胞增多的典型原因包括囊性肾病、肾脏或其他促红细胞生成素分泌肿瘤、高原肾综合征、促红细胞生成素刺激剂过量、雄激素治疗、大量吸烟、慢性肺病、阻塞性睡眠呼吸暂停、IgA 肾病、肾移植后红细胞增多、肾动脉狭窄和先天性病因。在排除红细胞增多症的常见获得性原因和/或存在提示性参数后，应考虑原发性红细胞增多症或真性红细胞增多症（PV），并对患者进行体细胞突变筛查。诱导红细胞增多症的最新实体与钠/葡萄糖协同转运蛋白 2 (SGLT2) 抑制剂的使用有关，该抑制剂假设可激活缺氧诱导因子 2α (HIF-2α)，并在某些情况下揭示 PV。本综述重点关注 PV 的发病机制、肾脏表现和治疗、SGLT2 抑制剂引起的红细胞增多的病理生理学以及及时对这些患者进行突变筛查的相关性。