Abstract：
A 66-year-old non-smoker presented with a 2-week history of new-onset pedal oedema and gross haematuria. On evaluation, he was found to be hypertensive and oedematous with a haemoglobin of 19.1 g/dl, platelet count of 546,000/mm3, and creatinine of 2.6 mg/dl. Urine examination revealed abundant RBCs with 3+ albumin on three separate occasions. His 24-h urine protein level was 3830 mg/day, with a serum cholesterol level of 303 mg/dl. Secondary erythrocytosis and thrombocytosis tests were negative. Bone marrow examination revealed hypercellularity, erythroid hyperplasia, tight clusters of large megakaryocytes, and megakaryocytic hyperplasia suggestive of polycythemia vera. PCR analysis revealed a JAK2V617 F (exon 14) mutation. In view of nephrotic syndrome, azotemia, and microscopic haematuria, a renal biopsy was performed, which revealed features of IgA nephropathy with advanced interstitial fibrosis and tubular atrophy. He was started on angiotensin receptor blockers with hydroxy urea as a part of treatment. This case report highlights the association of glomerular disease with polycythaemia vera and the need of prompt renal biopsy for diagnosis and management.
摘要：
一名66岁的非吸烟者，有2周的新发踏板水肿和肉眼血尿病史。关于评估，他被发现患有高血压和水肿，血红蛋白为19.1克/分升，血小板计数为546,000/mm3，肌酐为2.6mg/dl。尿液检查显示在三个不同的情况下富含3白蛋白的红细胞。他的24小时尿蛋白水平为3830毫克/天，血清胆固醇水平为303mg/dl。继发性红细胞增多症和血小板增多试验均为阴性。骨髓检查显示细胞增多，红系增生,紧密的大巨核细胞簇，巨核细胞增生提示真性红细胞增多症。PCR分析显示JAK2V617F(外显子14)突变。鉴于肾病综合征，氮质血症,和显微镜下的血尿，进行了肾活检,揭示了IgA肾病伴有晚期间质纤维化和肾小管萎缩的特征。他开始使用血管紧张素受体阻滞剂和羟基脲作为治疗的一部分。该病例报告强调了肾小球疾病与真性红细胞增多症的关联，以及需要及时进行肾活检以进行诊断和治疗。
