Amyloid Light-Chain (AL) Amyloidosis of the Trachea Associated With an Indolent B-cell Neoplasm

TL;DR

A rare case of tracheal amyloidosis linked to a B-cell neoplasm is described, highlighting diagnostic and treatment challenges.

Contribution

This case highlights the rare association between tracheal AL amyloidosis and indolent B-cell neoplasm, emphasizing diagnostic and therapeutic complexities.

Findings

Tracheal AL amyloidosis was diagnosed through bronchoscopy and biopsies in a patient with an underlying B-cell neoplasm.

The patient's treatment included rituximab, zanubrutinib, and dexamethasone, with ongoing outpatient follow-up.

The case underscores the rarity and management difficulties of tracheobronchial amyloidosis linked to B-cell malignancies.

Abstract

We report the case of a 66-year-old woman who was diagnosed with localized tracheal amyloid light-chain (AL) amyloidosis caused by an underlying B-cell neoplasm. The diagnosis was confirmed through subsequent bronchoscopy and biopsies; however, she experienced a challenging episode of hypoxic respiratory failure that required intervention. Repeat bronchoscopies showed persistent subglottic stenosis and tracheobronchomalacia, which led to tracheal debulking surgery and additional interventions. The patient's treatment began with rituximab, zanubrutinib, and dexamethasone with outpatient follow-up. The rarity of tracheobronchial amyloidosis and its connection to B-cell malignancies are highlighted, emphasizing the challenges in diagnosis and the importance of tailored treatment strategies. The patient's clinical course, characterized by atypical respiratory symptoms, delayed diagnosis, and an evolving treatment approach, underscores the complexities of managing such a rare and intricate case.

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