Nodular pulmonary amyloidosis with spontaneous fatal blood aspiration

• Two cases of fatal hemoptysis due to nodular pulmonary amyloidosis are presented.
• Histological examination revealed amyloid deposits within pulmonary blood vessels.
• Vascular amyloid depositions lead to fragility of blood vessels.
• As a result spontaneous hemorrhages cause massive blood aspiration.

Amyloidosis is a multisystem disease, which is characterized by the extracellular deposition of insoluble abnormal fibrils. Histological and subsequent immunohistochemical examinations are necessary for the determination of the diagnosis and the classification of the amyloid type. The most common systemic variant is immunoglobulin-derived light chain (AL) amyloidosis. However, local or organ-limited AL amyloidosis can occur. Isolated pulmonary amyloidosis is a rare condition and frequently an incidental finding at chest scans or during autopsy. Generally, it is associated with a benign prognosis.Here, we present two fatal cases, in which the cause of death was asphyxiation due to severe blood aspiration. During autopsy, several nodules were found in the lungs. Based on histological and immunohistochemical analysis, the diagnosis of an isolated nodular pulmonary AL amyloidosis lambda light chain was made. Amyloid was also present in pulmonary blood vessels, which lead to fragility and finally fatal hemorrhage.