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Adelita Vizcaino, Charles Eberhart, Fausto J. Rodriguez; Hemophagocytic lymphohistiocytosis in adults with intraocular involvement: Clinicopathologic features of 3 cases. Invest. Ophthalmol. Vis. Sci. 2017;58(8):1796.
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© ARVO (1962-2015); The Authors (2016-present)
Hemophagocytic lymphohistiocytosis (HLH) is an infrequent inflammatory multisystemic syndrome, and only rare cases with ophthalmic involvement describing their pathologic features have been previously reported. We performed a retrospective, observational clinical study to assess the clinicopathologic features in three adult-onset cases
Clinical information was obtained from retrospective review of the medical records, while systemic and ophthalmic postmortem findings were obtained from the final autopsy reports. All hematoxylin and eosin stained slides from the globes and optic nerves were reviewed. Immunostains for CD163, CD3, CD20 and BRAF p.V600E were performed
Three adult men – two with history of viral infection – developed persistent fever, fatigue, bone marrow abnormalities, and irreversible multiorgan failure. Visual impairment was also documented in two cases. Complete autopsies were performed. Ophthalmic pathology demonstrated a bilateral histiocytic infiltrate with scant lymphocytes affecting the uvea. Focal extension to the retina, optic nerve and trabecular meshwork were also identified, as well as hemophagocytosis in one case. Macrophages showed strong immunoreactivity for CD163 antibody and lacked BRAF p.V600E mutant protein
Histologic features of HLH in the eye are poorly documented, with prior reports restricted to children. Our three adult cases are reported using updated criteria, and despite the difference in age show changes similar to those in the pediatric population
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
Pathologic features of hemophagocytic lymphohistiocytosis with intraocular involvement (Case 3). Marked histiocytic infiltrate involving the choroid (arrow, A,B). Focal hemophagocytosis was identified (arrows, C), as well as involvement of the iris (D, arrows). Iron deposition was conspicuous (Prussian blue, E) and focal retinal involvement was identified through immunohistochemistry (CD163, F)
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