Pulmonary cystic disorder related to light chain deposition disease
Pulmonary cystic disorder related to light chain deposition disease
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DOI：
10.1164/rccm.200510-1620cr
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发表时间：
2006-04-01
影响因子：
24.7
通讯作者：
Danel, C
中科院分区：
文献类型：
--
作者：
Colombat, M;Stern, M;Danel, C
轻链沉积病（LCDD）是一种罕见的疾病，非常罕见地影响肺部。我们报告三例严重的囊性肺LCDD导致肺移植。这种情况以前从未报道过。这三个病人表现为进行性阻塞性肺模式，伴有大量囊肿弥漫性分布在双肺。该疾病的组织学特征是肺泡壁、小气道和血管中出现非淀粉样无定形沉积物。它与肺气肿样改变和小气道扩张有关。在异常沉积物上和沿着基底膜显示单型K轻链固定。电子显微镜显示粗颗粒状电子致密沉积物在相同的本地化。1例患者的唾液腺中发现轻度肺外沉积物。未发现免疫增殖性疾病。我们的结论是，LCDD可能主要影响肺，目前作为一个肺囊性疾病，并导致严重的呼吸功能不全。
Light chain deposition disease (LCDD) is a rare disorder that very uncommonly affects the lung. We report three cases of severe cystic pulmonary LCDD leading to lung transplantation. Such a presentation has never been previously reported. The three patients present with a progressive obstructive pulmonary pattern associated with numerous cysts diffusely distributed in both lungs. The disease was histologically characterized by non-amyloid amorphous deposits in the alveolar walls, the small airways and the vessels. It was associated with emphysematous-like changes and small airway dilation. Monotypic K light chain fixation was demonstrated on the abnormal deposits and along the basement membranes. Electron microscopy revealed coarsely granular electron-dense deposits in the same localizations. Mild extrapulmonary deposits were found in salivary glands in one patient. No immunoproliferative disorder was identified. We conclude that LCDD may primarily affect the lung, present as a pulmonary cystic disorder, and lead to severe respiratory insufficiency.