Kanoh T
Department of Internal Medicine, Faculty of Medicine, Kyoto University.
Rinsho Ketsueki. 1996 Nov;37(11):1293-6.
Pleural involvement in primary (AL) amyloidosis was reported. Pulmonary involvement in patients with primary amyloidosis is not so uncommon, but the pleura is regarded as an unusual site for amyloid deposition. A woman, born in 1942, was referred for evaluation of a four-month history of edema of eye lids and lower legs in 1987. Nephrotic syndrome with Bence Jones (lambda-type) proteinuria was demonstrated. She was diagnosed of having renal amyloidosis. In 1990, X-ray films incidentally demonstrated a small amount of right pleural effusion. A needle biopsy of the pleura was performed. Congo red stain demonstrated amyloid deposition in the pleura. The following clinical features indicating systemic amyloidosis occurred in succession: orthostatic hypotension, diarrhea or constipation, carpal tunnel syndrome, and weight loss. In 1993, sudden death due to cardiac arrest occurred. The patient survived 6 years after the diagnosis of renal amyloidosis. A combined use of melphalan, prednisolone, colchicine, and dimethyl sulfoxide (DMSO) might have contributed to the long survival of the patient. At postmortem examination, systemic deposition of type AL amyloid was confirmed. In patients with pleural effusion and multiorgan involvement or monoclonal gammopathy, a closed pleural biopsy should be performed, especially if the effusion is transudative.
有报道称原发性(AL)淀粉样变性可累及胸膜。原发性淀粉样变性患者出现肺部受累并不罕见,但胸膜被认为是淀粉样蛋白沉积的不常见部位。一名1942年出生的女性于1987年因眼睑和小腿水肿4个月前来接受评估。检查发现有伴本-周(λ型)蛋白尿的肾病综合征。她被诊断为肾淀粉样变性。1990年,X线片偶然显示有少量右侧胸腔积液。对胸膜进行了针吸活检。刚果红染色显示胸膜中有淀粉样蛋白沉积。随后相继出现了提示系统性淀粉样变性的以下临床特征:体位性低血压、腹泻或便秘、腕管综合征和体重减轻。1993年,患者因心脏骤停突然死亡。该患者在诊断为肾淀粉样变性后存活了6年。美法仑、泼尼松龙、秋水仙碱和二甲亚砜(DMSO)的联合使用可能有助于患者的长期存活。尸检时证实有AL型淀粉样蛋白的系统性沉积。对于有胸腔积液且有多器官受累或单克隆丙种球蛋白病的患者,应进行闭式胸膜活检,尤其是当积液为漏出液时。