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伴有肾血栓性微血管病的痛风石的罕见病因

"Unusual Cause Of Tophi With Renal Thrombotic Microangiopathy".

作者信息

Sahu Kamal Kant, Law Arjun Datt, Kumar Ganesh, Dhir Varun, Naseem Shano, Nada Ritambhra, Varma Subhash Chander, Malhotra Pankaj

机构信息

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012 India.

Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012 India.

出版信息

Indian J Hematol Blood Transfus. 2016 Jun;32(Suppl 1):100-3. doi: 10.1007/s12288-015-0539-9. Epub 2015 Apr 16.

Abstract

Chronic neutrophilic leukemia (CNL) is a rare entity amongst myeloproliferative neoplasms (MPNs). The classical presentation of CNL is with splenomegaly, mature neutrophilic leucocytosis and hyperuricemia. We herein report a case who presented with symptoms of acute gouty arthritis. Physical examination showed typical red, tender tophi in the right hand, right foot and both pinnae suggesting an acute episode of gout. During evaluation, moderate splenomegaly, mature neutrophilia, hyperuricemia and sub-nephrotic range range proteinuria were noted. Bone marrow examination and kidney biopsy was done. Final diagnosis of CNL with acute gouty arthritis and chronic renal thrombotic microangiopathy (TMA) was made. Although hyperuricemia is a common finding in MPNs but presentation of our case with symptoms of acute tophi and chronic TMA is atypical.

摘要

慢性中性粒细胞白血病(CNL)在骨髓增殖性肿瘤(MPN)中是一种罕见的疾病。CNL的典型表现为脾肿大、成熟中性粒细胞增多和高尿酸血症。我们在此报告一例表现为急性痛风性关节炎症状的病例。体格检查显示右手、右脚及双耳有典型的红色、压痛性痛风石,提示痛风急性发作。在评估过程中,发现有中度脾肿大、成熟中性粒细胞增多、高尿酸血症及亚肾病范围蛋白尿。进行了骨髓检查和肾脏活检。最终诊断为CNL合并急性痛风性关节炎和慢性肾血栓性微血管病(TMA)。虽然高尿酸血症在MPN中很常见,但我们的病例表现为急性痛风石症状和慢性TMA并不典型。

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