TY - JOUR
T1 - Ocular adnexal marginal zone lymphoma arising in a patient with immunoglobulin-G4-related ophthalmic disease
T2 - A 4-year delay in diagnosis
AU - Sabt, Buthaina
AU - Al Yahyai, Majda
AU - Al-Mujaini, Arwa
AU - Al-Mujaini, Abdullah
N1 - Publisher Copyright:
© 2021 Elsevier B.V.. All rights reserved.
PY - 2021/4/1
Y1 - 2021/4/1
N2 - Ocular adnexal marginal zone B cell lymphomas (MZBLs) make up the majority of lymphomas arising from the ocular adnexa. Immunoglobulin-G4 (IgG4)-related disease is a recently proposed entity with several unique clinicopathological features, such as enlargement of affected organs, elevated serum IgG4 level, and infiltration with IgG4-positive plasma cells. Ocular adnexal MZBLs are reported to arise in IgG4-related sclerosing dacryoadenitis, indicating a possible link between the two conditions. Here, we describe a 37-year-old Omani male who presented with right periorbital swelling and proptosis 4 years before presentation. He was diagnosed to have right orbital pseudotumor and exhibited good response to steroid therapy. However, 4 years later, rapid swelling of the right orbital mass was observed. The patient underwent lacrimal gland biopsy. Although the histology was consistent with IgG4-related disease, the infiltrating large atypical lymphoid cells showed that immunoglobulin light-chain restriction and dense lymphoplasmacytic infiltrate involving the soft tissue were seen. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells of the right lacrimal gland.
AB - Ocular adnexal marginal zone B cell lymphomas (MZBLs) make up the majority of lymphomas arising from the ocular adnexa. Immunoglobulin-G4 (IgG4)-related disease is a recently proposed entity with several unique clinicopathological features, such as enlargement of affected organs, elevated serum IgG4 level, and infiltration with IgG4-positive plasma cells. Ocular adnexal MZBLs are reported to arise in IgG4-related sclerosing dacryoadenitis, indicating a possible link between the two conditions. Here, we describe a 37-year-old Omani male who presented with right periorbital swelling and proptosis 4 years before presentation. He was diagnosed to have right orbital pseudotumor and exhibited good response to steroid therapy. However, 4 years later, rapid swelling of the right orbital mass was observed. The patient underwent lacrimal gland biopsy. Although the histology was consistent with IgG4-related disease, the infiltrating large atypical lymphoid cells showed that immunoglobulin light-chain restriction and dense lymphoplasmacytic infiltrate involving the soft tissue were seen. Consequently, he was diagnosed with extranodal marginal zone lymphoma with abundant IgG4-positive cells of the right lacrimal gland.
KW - Immunoglobulin-G4-related disease
KW - lymphoproliferative disorders
KW - marginal zone lymphoma
KW - ocular adnexa
KW - orbital pseudotumor
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U2 - 10.4103/1319-4534.337858
DO - 10.4103/1319-4534.337858
M3 - Article
C2 - 35391806
AN - SCOPUS:85126445482
SN - 1319-4534
VL - 35
SP - 164
EP - 166
JO - Saudi Journal of Ophthalmology
JF - Saudi Journal of Ophthalmology
IS - 2
ER -