Background: This article reports a case of primary localized conjunctival λ light-chain (AL) amyloidosis.
Methods: Case report.
Results: A 73-year-old woman presented with a 1-year history of a painless growth in the conjunctiva of the left eye. A yellow-salmon pink diffuse mass of tissue was identified in the inferomedial bulbar conjunctiva and inferior fornix. An incisional biopsy was performed. The histopathological and immunohistochemical examinations revealed interstitial and vascular amyloid deposits of λ light chains. The diagnosis was amyloidosis of the conjunctiva. The systemic evaluation revealed normal findings and systemic amyloidosis was excluded. Nevertheless, due to an unexplained cardiac insufficiency and after consultation with the treating hematologist a treatment with three cycles of systemic chemotherapy with melphalan and prednisolone was initiated but 6 months later the conjunctival mass in the inferior fornix showed persistence and complete excision was performed. At 16, 24 and 44 months of follow-up no evidence of recurrence was seen on clinical examination. The magnetic resonance imaging (MRI) at 16 and 24 months of follow-up showed no associated cranial or orbital infiltration.
Conclusion: Conjunctival AL amyloidosis is a rare clinical entity. Because of the heterogeneity of amyloidosis in clinical presentation, pattern of amyloid-related organ toxicity, association with lymphoproliferative diseases and rate of disease progression, identification of amyloid deposits is essential and systemic involvement has to be excluded.