Localized Laryngeal Amyloidosis Treated With Systemic Therapy: Case Series and Literature Review.
TL;DR
Two adult female patients with biopsy-proven localized laryngeal amyloidosis without evidence of systemic involvement who were treated with plasma cell-directed immunochemotherapy in collaboration with ENT and head and neck surgery teams are reported, highlighting a potential role for systemic plasma cell-targeted therapy.
Abstract
Localized laryngeal amyloidosis is a localized plasma cell-mediated process characterized by extracellular amyloid deposition confined to the larynx. Surgical excision has traditionally been the mainstay of treatment; however, complete resection may be anatomically difficult, and some patients experience persistent or progressive disease. Evidence supporting systemic therapy in localized disease remains limited. We report two adult female patients with biopsy-proven localized laryngeal amyloidosis without evidence of systemic involvement who were treated with plasma cell-directed immunochemotherapy in collaboration with ENT and head and neck surgery teams. Both patients presented with worsening dysphonia. One had persistent symptoms following partial surgical resection, while the other had multifocal unresectable supraglottic disease. Given limited surgical options, treatment with daratumumab, cyclophosphamide, bortezomib, and dexamethasone (Dara-CyBorD) was initiated. Therapy was well tolerated overall, with only mild neuropathy and mild leukopenia reported. After 6-14 cycles, both patients experienced marked improvement in voice quality and functional status, with no radiographic or clinical evidence of progression. Neither patient required additional surgical intervention, and both achieved durable symptom control during follow-up. These cases highlight a potential role for systemic plasma cell-targeted therapy in selected patients with localized laryngeal amyloidosis when surgery is noncurative or not feasible. Although local resection alone remains curative for many patients, multidisciplinary collaboration is essential in complex cases. Further study is needed to clarify patient selection, optimal treatment duration, and long-term outcomes of systemic immunochemotherapy in this rare disorder.