Ocular adnexal marginal zone lymphoma arising in a patient with IgG4-related ophthalmic disease

Kenji NishidaDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan,

Yuka SogabeDepartment of Ophthalmology, Mitoyo General Hospital, Kan-Onji, Japan,

Ayako MakiharaDepartment of Dermatology, Mitoyo General Hospital, Kan-Onji, Japan,

Akemi SenooDepartment of Dermatology, Mitoyo General Hospital, Kan-Onji, Japan,

Hisanori MorimotoDepartment of Internal Medicine, Mitoyo General Hospital, Kan-Onji, Japan, and

Mai TakeuchiDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan,

Yuka GionDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan,

Tadashi YoshinoDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan,

Yasuharu SatoDepartment of Pathology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan, ;Division of Pathophysiology, Okayama University Graduate School of Health Sciences, Okayama, JapanCorrespondence[email protected]

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Abstract

A 41-year-old man was diagnosed with immunoglobulin G4-related disease (IgG4-RD) in both eyelids 4 years ago and exhibited good response to steroid therapy. However, rapid swelling of the right eyelid lesion was recently observed. As IgG4-RD progression was suspected, biopsy was performed. Although the histology was consistent with IgG4-RD, the infiltrating large atypical lymphoid cells showed immunoglobulin light-chain restriction and IgH gene rearrangement. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells.

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Ocular adnexal marginal zone lymphoma arising in a patient with IgG4-related ophthalmic disease

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Ocular adnexal marginal zone lymphoma arising in a patient with IgG4-related ophthalmic disease

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