[Broncho-pulmonary amylosis (about two cases) (author's transl)]

Area of Science:

• Pulmonary Medicine
• Hematology
• Pathology

Background:

• Broncho-pulmonary amyloidosis is a rare condition characterized by amyloid deposition in the lungs.
• Monoclonal gammopathies, associated with abnormal immunoglobulin production, are increasingly recognized as a potential underlying cause.

Observation:

• Two cases of broncho-pulmonary amyloidosis with monoclonal gammopathies are presented.
• Case 1: Diffuse amyloidosis with bilateral pulmonary infiltration, pleural effusions, and IgG lambda monoclonal protein with Bence Jones proteinuria, leading to a fatal outcome due to renal insufficiency.
• Case 2: Localized amyloidosis associated with mediastino-pulmonary lymphoplasmocytic lymphoma secreting IgM lambda, resulting in death within months despite pneumonectomy.

Findings:

• Amyloidosis in both cases was confirmed through biopsies and post-mortem examinations.
• The underlying mechanism appears to be the abnormal synthesis of immunoglobulins, as elucidated by Glenner's work.
• The clinical presentation and diagnostic challenges of broncho-pulmonary amyloidosis are highlighted.

Implications:

• This study underscores the critical link between monoclonal gammopathies and broncho-pulmonary amyloidosis.
• Understanding the immunoglobulin nature of amyloid is key to diagnosing and potentially managing this rare pulmonary disease.
• Further research into the pathogenesis and therapeutic strategies for amyloidosis associated with plasma cell dyscrasias is warranted.