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一例轻链(AL)淀粉样变性伴心力衰竭、肾功能不全和肝素诱导的血小板减少症,成功接受了腹膜透析治疗。

A case of light chain (AL) amyloidosis with heart failure, renal dysfunction, and heparin-induced thrombocytopenia successfully treated with peritoneal dialysis.

机构信息

Division of Nephrology and Endocrinology, Faculty of Medicine, Tohoku Medical and Pharmaceutical University, 1-15-1 Fukumuro, Miyagino-ku, Sendai, Miyagi, 983-8536, Japan.

Division of Integrative Renal Replacement Therapy, Faculty of Medicine, Tohoku Medical and Pharmaceutical University, Sendai, Japan.

出版信息

CEN Case Rep. 2021 May;10(2):214-219. doi: 10.1007/s13730-020-00539-w. Epub 2020 Oct 28.

DOI:10.1007/s13730-020-00539-w
PMID:33113095
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8019447/
Abstract

A 65-year-old woman was hospitalized for heart failure and pneumonia in a nearby hospital. She had been previously diagnosed as light chain (AL) amyloidosis and treated with melphalan plus dexamethasone (Mel-Dex), and lenalidomide plus dexamethasone (Len-Dex). She started treatment including antimicrobials and diuretics, but her renal function worsened progressively, and she was transferred to our hospital for nephrological care. She was treated with antimicrobials, noradrenaline, dobutamine, and continuous hemodiafiltration. Her general condition gradually stabilized, and she was switched to intermittent hemodialysis (HD). However, HD was discontinued due to intradialytic hypotension and the development of heparin-induced thrombocytopenia. Her renal replacement therapy was switched to peritoneal dialysis (PD), which enabled good volume control and stable cardiac function. She was discharged and is still in good condition, without serious complications and achieving a considerably better prognosis than was predicted. Our case suggests that PD is an effective modality for patients with AL amyloidosis with heart failure and renal dysfunction.

摘要

一位 65 岁女性因心力衰竭和肺炎在附近医院住院。她先前被诊断为轻链(AL)淀粉样变性,并接受了美法仑联合地塞米松(Mel-Dex)和来那度胺联合地塞米松(Len-Dex)治疗。她开始接受包括抗生素和利尿剂在内的治疗,但肾功能逐渐恶化,因此被转至我们医院进行肾病治疗。她接受了抗生素、去甲肾上腺素、多巴酚丁胺和连续性血液透析滤过治疗。她的一般情况逐渐稳定,并转为间歇性血液透析(HD)。然而,由于透析中低血压和肝素诱导的血小板减少症的发展,HD 被停止。她的肾脏替代治疗改为腹膜透析(PD),这使她能够很好地控制容量并稳定心脏功能。她已出院,情况良好,没有严重并发症,预后明显好于预期。我们的病例表明,PD 是心力衰竭和肾功能不全的 AL 淀粉样变性患者的一种有效治疗方式。

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Clin Exp Nephrol. 2019 Nov;23(11):1315-1322. doi: 10.1007/s10157-019-01773-y. Epub 2019 Aug 19.
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Efficacy and Biocompatibility of Neutral Icodextrin Peritoneal Dialysis Fluid.中性艾考糊精腹膜透析液的疗效与生物相容性
Adv Perit Dial. 2016;32:46-50.
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Plasma cell neoplasm as a risk factor for early thrombosis of arteriovenous fistula.浆细胞肿瘤作为动静脉内瘘早期血栓形成的一个危险因素。
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Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response.轻链型心脏淀粉样变性:促进早期诊断及心脏反应的策略
Heart. 2017 Jul;103(14):1065-1072. doi: 10.1136/heartjnl-2016-310704. Epub 2017 Apr 29.
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Systemic amyloidosis.系统性淀粉样变性。
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Recent advances in the management of AL Amyloidosis.AL淀粉样变性病治疗的最新进展
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Natural history and therapy of AL cardiac amyloidosis.AL型心脏淀粉样变性的自然病史与治疗
Heart Fail Rev. 2015 Mar;20(2):155-62. doi: 10.1007/s10741-014-9464-5.
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