Waldenström巨球蛋白血症诱导的心脏淀粉样蛋白轻链淀粉样变性。

IF 1.3 Q2 MEDICINE, GENERAL & INTERNAL
Jerry Fan, Michael Chukwu, Corry B Sanford, Deborah Jebakumar, Nicholas Quitoriano, Vinh Nguyen
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引用次数: 0

摘要

背景:Waldenström巨球蛋白血症是一种罕见的浆细胞癌,其特征是免疫球蛋白M (IgM)的过量产生。igm相关的系统性淀粉样蛋白轻链(AL)淀粉样变性是Waldenström巨球蛋白血症的一种罕见并发症,其特征是沉积在包括心脏在内的各种器官中的λ轻链的错误折叠。我们描述了一例继发于Waldenström巨球蛋白血症和igm相关AL淀粉样变的进行性非缺血性心肌病,药物治疗难愈,并强调了诊断和管理方面的挑战。病例报告:一名64岁男性高血压患者出现心力衰竭症状。诊断检查显示有Waldenström巨球蛋白血症和igm相关的系统性AL淀粉样变性影响心脏。进一步的调查证实了Waldenström巨球蛋白血症的存在,骨髓中有lambda限制性淋巴浆细胞浸润。肾活检显示淀粉样肾病,心内膜活检显示广泛的纤维物质沉积,与心脏淀粉样变性一致。由于病人病情晚期且身体虚弱,我们决定将重点放在安宁疗护的舒适疗护上。结论:Waldenström巨球蛋白血症引起的心脏AL淀粉样变是一种具有挑战性的临床情况,其特征是两种不同的血液系统疾病共存影响心功能。诊断需要综合评估,管理需要针对Waldenström巨球蛋白血症和心脏淀粉样变性的多学科方法。需要进一步的研究和合作,以改进诊断技术,改进治疗方法,并提高这种罕见和复杂疾病的患者预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Waldenström Macroglobulinemia-Induced Cardiac Amyloid Light Chain Amyloidosis.

Background: Waldenström macroglobulinemia is a rare cancer of plasma cells characterized by the excessive production of immunoglobulin M (IgM). IgM-associated systemic amyloid light chain (AL) amyloidosis is a rare complication of Waldenström macroglobulinemia, characterized by the misfolding of lambda light chains that deposit in various organs, including the heart. We describe a case of progressive nonischemic cardiomyopathy secondary to Waldenström macroglobulinemia and IgM-associated AL amyloidosis that was refractory to medical therapy and highlight the challenges in diagnosis and management. Case Report: A 64-year-old male with hypertension presented with symptoms of heart failure. Diagnostic workup revealed evidence of Waldenström macroglobulinemia and IgM-associated systemic AL amyloidosis affecting the heart. Further investigations confirmed the presence of Waldenström macroglobulinemia with lambda-restricted lymphoplasmacytic infiltrate in the bone marrow. Renal biopsy revealed amyloid nephropathy, and endomyocardial biopsy showed extensive deposits of fibrillary material consistent with cardiac amyloidosis. Because of the patient's advanced disease state and frailty, the decision was made to focus on comfort care with hospice. Conclusion: Waldenström macroglobulinemia-induced cardiac AL amyloidosis is a challenging clinical scenario characterized by the coexistence of 2 distinct hematologic disorders impacting cardiac function. Diagnosis requires a comprehensive evaluation, and management necessitates a multidisciplinary approach targeting both Waldenström macroglobulinemia and cardiac amyloidosis. Further research and collaboration are needed to improve diagnostic techniques, refine treatment approaches, and enhance patient outcomes for this rare and complex condition.

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来源期刊
Ochsner Journal
Ochsner Journal MEDICINE, GENERAL & INTERNAL-
CiteScore
2.10
自引率
0.00%
发文量
71
审稿时长
24 weeks
期刊介绍: The Ochsner Journal is a quarterly publication designed to support Ochsner"s mission to improve the health of our community through a commitment to innovation in healthcare, medical research, and education. The Ochsner Journal provides an active dialogue on practice standards in today"s changing healthcare environment. Emphasis will be given to topics of great societal and medical significance.
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