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.

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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