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2018 ; 32
(1
): 56-61
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Orbital progressive transformation of germinal centers as part of the spectrum of
IgG4-related ophthalmic disease: Clinicopathologic features of three cases
#MMPMID29755273
Vizcaino MA
; Joseph SS
; Eberhart CG
Saudi J Ophthalmol
2018[Jan]; 32
(1
): 56-61
PMID29755273
show ga
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 of these PTGC in
extra-nodal locations, including the orbit, may provide a better understanding of
the histologic spectrum of this disease.