Abstract:
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 JAK2V617F 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 JAK2 mutation screening in these patients.
摘要:
红细胞增多症或红细胞增多症定义为红细胞浓度高于年龄和性别特定的正常水平。与慢性肾脏病(CKD)患者中非常常见的贫血不同,红细胞增多症的频率较低,但需要医疗保健专业人员的具体理解才能提供最佳护理。红细胞增多症,尤其是在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相关的红细胞增多症的经典原因包括囊性肾病,肾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症,肾动脉狭窄和先天性病因。在排除了红细胞增多症的常见获得性原因后,和/或在存在暗示性参数的情况下,应考虑原发性红细胞增多症或真性红细胞增多症(PV),并筛查JAK2V617F体细胞突变患者.诱导红细胞增多的最新实体与使用钠-葡萄糖协同转运蛋白-2(SGLT2)抑制剂有关,该抑制剂假设激活缺氧诱导因子2-α(HIF-2α),并且在某些情况下揭开PV。本文就其发病机制进行综述,PV的肾脏表现和管理,SGLT2抑制剂诱导的红细胞增多的病理生理学和及时进行JAK2突变筛查的相关性.