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华氏巨球蛋白血症患者出现假血栓沉积伴微小病变型肾病综合征和慢性肾脏病:一例报告

Pseudothrombus deposition accompanied with minimal change nephrotic syndrome and chronic kidney disease in a patient with Waldenström's macroglobulinemia: A case report.

作者信息

Mwamunyi Mercy Julian, Zhu Hong-Yan, Zhang Chun, Yuan Ya-Pei, Yao Li-Jun

机构信息

Department of Nephrology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.

Department of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.

出版信息

World J Clin Cases. 2019 Aug 26;7(16):2393-2400. doi: 10.12998/wjcc.v7.i16.2393.

Abstract

BACKGROUND

Waldenström's macroglobulinemia (WM) is a rare lymphoid neoplasia, which can have renal complications. These rarely occur, and most common renal manifestations are mild proteinuria and microscopic hematuria. Herein we describe a case of WM that presented with pseudothrombi depositing in capillaries associated with minimal change nephrotic syndrome and chronic kidney disease (CKD).

CASE SUMMARY

A 52-year-old man presented with features suggesting nephrotic syndrome. Extensive workups were done, and there were elevated serum levels of interleukin-6 and vascular endothelial growth factor (VEGF), capillary pseudothrombus accumulation associated with minimal change nephrotic syndrome, CKD, and WM. Treatment was directed at the patient's WM with bortezomib, thalidomide, and dexamethasone whereby serum immunoglobulin M (IgM) decreased. The damage of IgM on the kidney was corrected; thus, the patient's proteinuria and serum creatinine had improved. The patient is still under clinical follow-up.

CONCLUSION

It is essential for clinicians to promptly pay more attention to patients presenting with features of nephrotic syndrome and do extensive workups to come up with a proper therapy strategy.

摘要

背景

华氏巨球蛋白血症(WM)是一种罕见的淋巴样肿瘤,可出现肾脏并发症。这些并发症很少见,最常见的肾脏表现是轻度蛋白尿和镜下血尿。在此,我们描述一例WM患者,其出现与微小病变肾病综合征和慢性肾脏病(CKD)相关的毛细血管内假血栓沉积。

病例摘要

一名52岁男性表现出提示肾病综合征的特征。进行了全面检查,发现血清白细胞介素-6和血管内皮生长因子(VEGF)水平升高,存在与微小病变肾病综合征、CKD和WM相关的毛细血管假血栓积聚。采用硼替佐米、沙利度胺和地塞米松针对患者的WM进行治疗,血清免疫球蛋白M(IgM)降低。IgM对肾脏的损害得到纠正,患者的蛋白尿和血清肌酐有所改善。该患者仍在临床随访中。

结论

临床医生必须及时更加关注表现出肾病综合征特征的患者,并进行全面检查以制定合适的治疗策略。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a34b/6718796/f29c17b757f6/WJCC-7-2393-g001.jpg

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