Orbital progressive transformation of germinal centers as part of the spectrum of IgG4-related ophthalmic disease: Clinicopathologic features of three cases

Saudi Journal of Ophthalmology : Official Journal of the Saudi Ophthalmological Society
M Adelita VizcainoCharles G Eberhart

Abstract

Progressive transformation of germinal centers (PTGC) is a form of follicular hyperplasia recently associated with immunoglobulin G4-related disease (IgG4-RD), but the ophthalmic manifestations of this combination are poorly described. In this retrospective case series, we present three cases of IgG4-related orbital disease (IgG4-ROD) showing varying degrees of PTGC involving the orbit and lacrimal gland. Three adult women presented with ill-defined lacrimal gland enlargement. Histologic sections showed variable fibrosis and large, irregular lymphoid follicles with prominent mantle zones penetrating the germinal centers, highlighted by Bcl-2 and/or IgD immunostains. The interfollicular areas contained a mixture of plasma cells, scattered histiocytes and eosinophils. Mixed T and B-cells were present, and no signs of monoclonality were identified. All cases showed more than 100 IgG4 positive cells per high power field. Epstein-Barr virus in situ hybridization performed in one case was negative. The serum IgG4 level was tested in one case and showed elevation above the normal range. After 2-10 months of follow-up, the patients showed either near-complete resolution or no remaining signs of ophthalmic disease. Increasing awareness ...Continue Reading

References

Nov 15, 2011·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Kate E GrimmDennis P O'Malley
Apr 7, 2012·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Yasuharu SatoTadashi Yoshino
Jul 14, 2012·The British Journal of Ophthalmology·Sepideh Amin, Alan Ramsay
Jan 5, 2014·Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Für Klinische Und Experimentelle Ophthalmologie·Yuka SogabeTetsuya Nakamura
Apr 8, 2014·The American Journal of Surgical Pathology·Mai TakeuchiTadashi Yoshino
Nov 14, 2014·Japanese Journal of Ophthalmology·Hiroshi GotoUNKNOWN Japanese Study Group for IgG4-Related Ophthalmic Disease
Feb 22, 2016·Seminars in Diagnostic Pathology·Kaustubh Mulay, Mark R Wick

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Citations

May 15, 2018·Saudi Journal of Ophthalmology : Official Journal of the Saudi Ophthalmological Society·J G Heathcote

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Methods Mentioned

BETA
Biopsy
PCR

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