Concurrence of orbital immunoglobulin G4-related disease and lymphoproliferative neoplasms: case series and review of literature

Theme: Oculoplastic & reconstructive surgery
What: Oculoplastic & reconstructive surgery
Part of: Oculoplastics II: Cutting edge research in oculoplastics! / Oculoplastie II: Recherche de pointe en oculoplastie!
When: 5/31/2024, 02:00 PM - 03:30 PM
Where: Room | Salle 714 A

Abstract

Purpose: Lymphoproliferative disorders span a wide spectrum of diseases ranging from benign infiltrative conditions to malignant lymphomas. Immunoglobulin G4-related disease (IgG4-RD) is an emerging immune-mediated systemic condition consisting of lymphoplasmacytic infiltrates and lesions with IgG4-positive plasma cells. Lymphoproliferative neoplasms, including lymphoma, may mimic IgG4-RD. There exists a growing interest in the relationship between IgG4-RD and lymphoproliferative neoplasms.

Study design: Retrospective observational case series and associated literature review 

Methods: Patient charts were reviewed. The National Library of Medicine (PubMed) database was employed to identify cases of concurrent orbital lymphoproliferative neoplasm and IgG4-RD and associated mechanistic studies.

 Results: Case 1 describes a 78-year-old man with a 3-month history of binocular diplopia and decreased vision in the left eye. Serum IgG4 levels were elevated. CT imaging of the orbits demonstrated diffuse enlargement and heterogenous enhancement of the left lacrimal gland and enlargement of both infraorbital nerves. The left lacrimal gland was biopsied, with histopathology showing extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT lymphoma) and concurrent IgG4+ plasma cell elevation. Case 2 involves a 38-year-old man with a 6-year history of progressive eyelid swelling and past medical history notable for cutaneous low grade B cell lymphoma. Bloodwork was notable for elevated serum IgG4 and immunohistochemistry of the biopsy tissue revealed sheets of IgG4+ monoclonal plasma cells and marked deposition of amyloid. Although IgG4-RD and lymphoma have distinct histopathological features and diagnostic criteria, they are both lymphoproliferative disorders and several reports have proposed a possible link between these two entities. T-helper-2 (Th2) and regulatory T-cell cytokines have been shown to be upregulated in IgG4-RD lesions. Moreover, Th2 and Treg cytokines were shown to be upregulated in ocular IgG4-associated marginal zone lymphoma when compared to non-IgG4-RD marginal zone lymphomas.

Conclusions: Herein, we present two cases of concurrent orbital IgG4-RD and lymphoproliferative neoplasm. These cases emphasize the importance of accurate histopathological tissue analysis and highlight the potential interplay between these two lymphoproliferative disorders. While the mechanistic link between IgG4-RD and lymphoma, if any, remains unknown, it is postulated that upregulation of regulatory T cells in IgG4-RD, which can promote tumor development through inhibition of anti-tumor immunity, may contribute to development of lymphoma.

Presenter(s)

Presenting Author: Sonia Anchouche

Additional Author(s):

Kenneth Chang, Department of Ophthalmology & Vision Sciences, University of Toronto, Toronto, Canada

Carlo Hojilla, Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada

Georges Nassrallah, Department of Ophthalmology & Vision Sciences, University of Toronto, Toronto, Canada

Navdeep Nijhawan, Department of Ophthalmology & Vision Sciences, University of Toronto, Toronto, Canada

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