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原发性系统性淀粉样变性和持续性胸腔积液。

Primary systemic amyloidosis and persistent pleural effusions.

机构信息

Alpert School of Medicine, Brown University, Providence, Rhode Island, USA.

出版信息

Amyloid. 2009 Dec;16(4):239-42. doi: 10.3109/13506120903421900.

Abstract

Persistent pleural effusions are not common in patients with primary systemic amyloidosis (AL). A recent review of this complication of the disease hypothesized that the pathophysiology of these effusions is pleural amyloid deposition, disrupting lymphatic drainage. We report the case of a 73-year-old woman with primary systemic AL and persistent bilateral pleural effusions, refractory to diuresis and repeated thoracenteses. The patient's cardiac and renal dysfunction was not severe enough to explain these persistent effusions. Thus, despite a lack of biopsy-proven amyloid deposition, we suggest that these effusions may be secondary to pleural amyloid deposition.

摘要

原发性系统性淀粉样变(AL)患者中持续性胸腔积液并不常见。近期对该疾病并发症的一项回顾性研究假设,这些胸腔积液的病理生理学是胸腔淀粉样物质沉积,破坏了淋巴引流。我们报告了一例 73 岁女性原发性系统性 AL 合并持续性双侧胸腔积液,利尿和反复胸腔穿刺均无效。该患者的心肾功能不全尚未严重到足以解释这些持续性胸腔积液。因此,尽管缺乏经活检证实的淀粉样物质沉积,但我们认为这些胸腔积液可能继发于胸腔淀粉样物质沉积。

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