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A Case of Essential Thrombocythemia and IgA Nephropathy with Literature Review of the Concurrence

机译:原发性血小板增多症和IgA肾病一例并发文献复习

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摘要

Myeloproliferative neoplasms such as essential thrombocythemia (ET) have been associated with glomerular disease on rare instances. A case of ET associated with immunoglobulin A nephropathy (IgAN) is described in a 57-year-old man with a history of hypertension. Progressively worsening renal function was noted in the patient along with unexplained mild thrombocytosis. Pathological review of renal biopsy identified IgAN concurrently with newly diagnosed JAK2-mutated ET. The patient was started on aspirin therapy and closely monitored for his renal function. A literature review of the association of ET and renal disease revealed nine cases of ET associated with IgAN, focal segmental glomerulosclerosis, and fibrillary glomerulonephritis. Comparison of the pathological features of the renal biopsies within the cases noted mesangial proliferation as a common finding, which has been described to be potentiated by platelet-derived growth factor (PDGF). This commonality may represent a link between ET and glomerular disease which deserves further attention in future cases. Improved management of such cases depends on the recognition of the combined occurrence of ET and glomerular diseases and uncovering the shared pathogenesis between platelets and glomeruli.
机译:在少数情况下,骨髓增生性肿瘤(例如原发性血小板增多症(ET))与肾小球疾病有关。一名患有高血压病的57岁男性描述了与免疫球蛋白A肾病(IgAN)相关的ET病例。病人的肾功能逐渐恶化,并伴有无法解释的轻度血小板增多症。肾脏活检的病理学检查同时鉴定了IgAN和新诊断的JAK2突变ET。患者开始接受阿司匹林治疗,并密切监测其肾功能。关于ET与肾脏疾病的关联的文献综述显示,有9例ET与IgAN,局灶性节段性肾小球硬化和原发性肾小球肾炎有关。病例中肾脏活检组织病理学特征的比较表明,肾小球系膜增生是一个常见发现,已被描述是由血小板衍生生长因子(PDGF)增强。这种共性可能代表了ET和肾小球疾病之间的联系,在未来的病例中值得进一步关注。改善此类病例的治疗取决于对ET和肾小球疾病合并发生的认识,并揭示血小板与肾小球之间的共同发病机制。

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