Subscribe to RSS
DOI: 10.1055/a-2761-3113
Progressive Bilateral Localised Amyloidosis of the Eyelids and Conjunctiva Four Years after MALT Lymphoma: A Case Report
Progressive bilaterale lokalisierte Amyloidose der Augenlider und Bindehaut 4 Jahre nach MALT-Lymphom: ein FallberichtAuthors
Introduction
Amyloidosis refers to a heterogeneous group of disorders characterized by the deposition of insoluble protein fibrils in various tissues, including the eyes and their adnexa. It can occur either systemically or locally and is classified based on the type of amyloid protein involved. To date, 42 different amyloid fibril proteins have been identified; however, not all are associated with ocular deposition [1].
Orbital and adnexal amyloidosis is rare and most commonly affects the eyelid, orbit, and conjunctiva. While it can be the initial manifestation of systemic involvement, most cases are localized with amyloid light-chain (AL) deposits being the most common, resulting from the accumulation of misfolded immunoglobulin light chains [2], [3]. Due to the heterogeneity of the disease and the limited literature available, the diagnosis and management of orbital and adnexal amyloidosis remain challenging.
Publication History
Received: 06 October 2025
Accepted: 02 December 2025
Article published online:
04 February 2026
© 2026. Thieme. All rights reserved.
Georg Thieme Verlag KG
Oswald-Hesse-Straße 14, 70469 Stuttgart, Germany
-
References
- 1 Buxbaum JN, Eisenberg DS, Fändrich M. et al. Amyloid nomenclature 2024: update, novel proteins, and recommendations by the International Society of Amyloidosis (ISA) Nomenclature Committee. Amyloid 2024; 31: 249-256
- 2 Banerjee P, Alam MS, Subramanian N. et al. Orbital and adnexal amyloidosis: Thirty years experience at a tertiary eye care center. Indian J Ophthalmol 2021; 69: 1161-1166
- 3 Dammacco R, Merlini G, Lisch W. et al. Amyloidosis and Ocular Involvement: an Overview. Semin Ophthalmol 2020; 35: 7-26
- 4 Zucca E, Conconi A, Pedrinis E. et al. Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood 2003; 101: 2489-2495
- 5 Mehta N, Schöder H, Chiu A. et al. Adnexal mass secondary to extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) with associated amyloid deposition. BMJ Case Rep 2014; 2014: bcr2014206699
- 6 Bruce CN, Kroft SH, Harris GJ. Orbital MALT lymphoma with amyloid deposition. Orbit 2024; 43: 526-530
- 7 Chen X, Wei M, Mou P. et al. The clinical outcomes of ultra-low-dose radiotherapy for primary conjunctival mucosa-associated lymphoid tissue lymphoma. Int Ophthalmol 2025; 45: 269
- 8 Matsuo Y, Monden Y, Sasaki K. et al. Treatment Outcomes in Patients with Conjunctival Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma. Clin Ophthalmol 2024; 18: 1999-2007
- 9 Prager AJ, Habib LA, Gambogi T. et al. Long-Term Follow-Up of 4 Patients with Conjunctival Amyloidosis. Ocul Oncol Pathol 2018; 4: 313-317
- 10 Liu Y, Li Y, Zhang C. et al. Efficacy and safety of lenalidomide in the treatment of B-cell non-Hodgkin lymphoma. Discov Oncol 2024; 15: 105
