Orbital lymphomas in autoimmune diseases

Vasileios Ragos¹, Stylianos Mastronikolis², Christoforos Asproudis³, Ioannis Stavrakis⁴, Anna Batistatou⁵, Despoina Spyropoulou⁶, Panagiotis Fotiades⁷, Dimitrios Peschos⁸, Ioannis Asproudis¹, Maria Adamopoulou⁹, Evangelos Tsiambas¹⁰, Nicholas Mastronikolis¹¹

¹Department of Maxillofacial Surgery, Medical School, University of Ioannina, Ioannina, Greece;

²Medical School, University of Heraklion, Crete, Greece;

³Department of Ophthalmology, University of Ioannina, Ioannina, Greece;

⁴ENT Department, “Sotiria” General Hospital, Athens, Greece;

⁵Department of Pathology, Medical School, University of Ioannina, Ioannina, Greece;

⁶Department of Radiation Oncology, Medical School, University of Patras, Greece;

⁷Department of Surgery, 424 General Army Hospital, Thessaloniki, Greece;

⁸Department of Physiology, Medical School, University of Ioannina, Greece;

⁹Department of Biomedical Sciences, University of West Attica, Greece;

¹⁰Department of Cytology, 417 VA (NIMTS) Hospital, Athens, Greece;

¹¹ENT Department, Medical School, University of Patras, Greece.

Summary

Primary orbital lymphomas (OL) represent a rare sub-category of non-Hodgkin’s lymphomas, but they constitute a significant proportion of ocular adnexal lymphomas. The majority of these lymphomas are of the NHL B-cell type. Histopathologically, OLs are characterized as extra-nodal marginal zone B-cell lymphoma, diffuse large B-cell lymphoma, mantle cell lymphoma, and follicular lymphoma. Diffuse large B-cell lymphoma and mantle cell lymphoma are high-grade lymphomas, while extra-nodal marginal zone B-cell lymphoma and follicular lymphoma are recognized as low-grade. Limited data exist regarding the relationship between OLs and other diseases. In this review, we focus on the association between OL and autoimmune diseases, such as Sjögren’s syndrome, Hashimoto’s thyroiditis, and myasthenia gravis.

Keywords: orbit, lymphoma, autoimmune diseases.

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