Delaey P, Plawny L, Nchimi A, Hirschi S, Weingertner N, Santelmo N, Wirtz G
Département de médecine interne, cliniques universitaires Saint-Luc, université catholique de Louvain, Bruxelles, Belgique; Service de pneumologie, centre hospitalier de Luxembourg, Strassen, Luxembourg.
Service d'hématologie, centre hospitalier de Luxembourg, Strassen, Luxembourg.
Rev Mal Respir. 2020 Feb;37(2):180-186. doi: 10.1016/j.rmr.2020.01.001. Epub 2020 Jan 31.
Light chain deposition disease is a rare anatomo-clinical disorder, which rarely leads to cystic lung destruction.
We report the case of a 62years old female patient with a history of a monoclonal gammopathy of unknown significance who developed progressive dyspnea. Thoracic CT-scan demonstrated a diffuse pulmonary cystic disorder with predominance in the right lower lobe. Thoracoscopic surgical resection of that lobe led to a diagnosis of non-amyloid kappa light chain deposits. Surgery also resulted in a lung volume reduction effect with clinical and functional benefits related to improved ventilation of adjacent segments.
This report of pulmonary cystic disorder related to a light chain deposition disease highlights the potential clinical and functional benefits observed after lung volume reduction surgery.
轻链沉积病是一种罕见的解剖学临床疾病,很少导致肺囊性破坏。
我们报告一例62岁女性患者,有意义未明的单克隆丙种球蛋白病病史,出现进行性呼吸困难。胸部CT扫描显示弥漫性肺囊性疾病,以下右肺叶为主。对该肺叶进行胸腔镜手术切除后诊断为非淀粉样κ轻链沉积。手术还产生了肺容积减少效应,对相邻肺段通气改善有临床和功能益处。
本例与轻链沉积病相关的肺囊性疾病报告突出了肺容积减少手术后观察到的潜在临床和功能益处。