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快速进展性肾衰竭患者凝血功能障碍的罕见病因

A Rare Cause of Coagulopathy in a Patient with Rapidly Progressive Renal Failure.

作者信息

Prabakaran Rudreshwar, Sethi Jasmine, Rathi Manish, Kohli Harbir Singh, Malhotra Pankaj, Gupta Krishan Lal

机构信息

Department of Nephrology, PGIMER, Chandigarh, India.

Department of Internal Medicine, PGIMER, Chandigarh, India.

出版信息

Indian J Nephrol. 2020 Mar-Apr;30(2):110-112. doi: 10.4103/ijn.IJN_213_18. Epub 2020 Feb 7.

Abstract

Deranged coagulogram is a common problem, which a nephrologist faces before doing a renal biopsy. We describe a rare cause of coagulopathy in a patient with rapidly progressive renal failure due to acquired factor X deficiency caused by systemic light chain amyloidosis (AL). The patient had prolonged prothrombin and activated partial thromboplastin time, which got corrected on mixing with normal plasma, and factor X activity was markedly reduced at 5%. Rectal biopsy and immunofixation electrophoresis established the diagnosis of AL and the patient was started on bortezomib-based chemotherapy. Hence, appropriate coagulation work-up should be conducted in patients with renal dysfunction with prolonged coagulation times, as it can sometimes reveal the underlying diagnosis in situations where renal biopsy could not be done due to high risk of bleeding.

摘要

凝血象异常是肾病学家在进行肾活检前常见的问题。我们描述了一例罕见的凝血病病因,该患者因系统性轻链淀粉样变性(AL)导致获得性X因子缺乏,进而出现快速进展性肾衰竭。患者的凝血酶原时间和活化部分凝血活酶时间延长,与正常血浆混合后可纠正,且X因子活性显著降低至5%。直肠活检和免疫固定电泳确诊为AL,患者开始接受基于硼替佐米的化疗。因此,对于凝血时间延长的肾功能不全患者,应进行适当的凝血检查,因为在因出血风险高而无法进行肾活检的情况下,凝血检查有时可揭示潜在诊断。

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本文引用的文献

6
Factor-X deficiency in amyloidosis: a critical review.
Am J Hematol. 1981 Dec;11(4):443-50. doi: 10.1002/ajh.2830110414.

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