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肺动脉高压作为淀粉样变性和冒烟型多发性骨髓瘤病例中的主要临床表现。

Pulmonary hypertension as a dominant clinical picture in a case of amyloidosis and smoldering multiple myeloma.

作者信息

Lehtonen Jukka, Kettunen Pasi

出版信息

Int J Cardiol. 2007 Jan 31;115(1):e29-30. doi: 10.1016/j.ijcard.2006.07.059. Epub 2006 Oct 17.

DOI:10.1016/j.ijcard.2006.07.059
PMID:17049643
Abstract

A 48-year-old male patient presented with dyspnea on exertion. Patient was found to have pulmonary hypertension. Myocardial biopsy showed amyloidosis and further work-up revealed Salmon-Durie stage 1A multiple myeloma. Patient had no other clinical manifestations of amyloidosis. It is possible that the pulmonary hypertension is caused by amyloid deposition into pulmonary arteries as the arterial amyloid deposition is common in AL amyloidosis. Treatment with sildenafil led to hemodynamic and symptomatic improvement.

摘要

一名48岁男性患者出现劳力性呼吸困难。患者被诊断为肺动脉高压。心肌活检显示为淀粉样变性，进一步检查发现为Salmon-Durie 1A期多发性骨髓瘤。患者无淀粉样变性的其他临床表现。肺动脉高压可能是由于淀粉样蛋白沉积于肺动脉所致，因为动脉淀粉样蛋白沉积在AL型淀粉样变性中很常见。使用西地那非治疗后，血流动力学和症状均有改善。

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