A Rare Case of Tracheal Amyloidosis in a Middle-Aged Person

TL;DR

This paper presents a rare case of tracheal amyloidosis in a 71-year-old man, highlighting the diagnostic challenges and the need for a multidisciplinary approach.

Contribution

The paper contributes a detailed clinical case of tracheal amyloidosis associated with a lymphoproliferative disorder, emphasizing diagnostic and management complexities.

Findings

Tracheal amyloidosis was diagnosed via CT and bronchoscopy in a middle-aged patient.

Biopsy confirmed amyloid deposition linked to a low-grade lymphoproliferative disorder.

The case underscores the importance of multidisciplinary care for effective diagnosis and treatment.

Abstract

Tracheal amyloidosis is a rare condition characterized by the extracellular deposition of misfolded proteins, primarily immunoglobulin (Ig) amyloid light chain (AL), within the tracheobronchial tree. Clinical manifestations include cough, exertional dyspnea, wheezing, hoarseness, chest tightness, and hemoptysis. Diagnosis involves imaging, such as CT of the neck and thorax, followed by histological confirmation via flexible bronchoscopy. Treatment is tailored to individual symptom severity. We present the case of a 71-year-old male patient who presented with progressive shortness of breath over six months. A CT thorax revealed mediastinal, subcarinal, and hilar lymphadenopathy, alongside high-grade stenosis of the left upper lobe bronchus. Endobronchial biopsy identified acellular material exhibiting apple-green birefringence under polarized light, consistent with amyloid deposition. Further excisional biopsy from the left inguinal region revealed a low-grade lymphoproliferative disorder with marked plasmacytic differentiation, contributing to AL amyloid production. The patient is currently receiving treatment for low-grade lymphoma under the supervision of a hematology consultant. This case highlights the diagnostic challenge posed by tracheal amyloidosis, emphasizing the importance of a multidisciplinary approach for effective diagnosis and management. Early recognition and comprehensive evaluation are essential for timely intervention, particularly in cases associated with underlying lymphoproliferative disorders.

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