Supplementary Material for: Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases
Vizcaino M.A.
Eberhart C.G.
Rodriguez F.J.
10.6084/m9.figshare.5057470.v1
https://karger.figshare.com/articles/journal_contribution/Supplementary_Material_for_Hemophagocytic_Lymphohistiocytosis_in_Adults_with_Intraocular_Involvement_Clinicopathologic_Features_of_3_Cases/5057470
<p><b><i>Background/Aims:</i></b> Hemophagocytic lymphohistiocytosis
(HLH) is an infrequent inflammatory multisystemic syndrome. Only rare
cases with ophthalmic involvement describing their pathologic features
have been previously reported. <b><i>Methods:</i></b> We report 3 cases of adult-onset HLH with bilateral ocular involvement and describe their clinicopathologic features. <b><i>Results:</i></b>
Three adult males - 2 with a history of viral infection - developed
persistent fever, fatigue, bone marrow abnormalities, and irreversible
multiorgan failure. Visual impairment was also documented in 2 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 1 case.
Macrophages showed strong immunoreactivity for CD163 antibody and lacked
BRAF p.V600E mutant protein. <b><i>Conclusion:</i></b> HLH is an
unusual disorder associated with several systemic conditions. Histologic
features in the eye are poorly documented, with prior reports
restricted to children. Our 3 adult cases are reported using updated
criteria and, despite the difference in age, show changes similar to
those observed in the pediatric population.</p>
2017-06-01 09:29:15
Hemophagocytic lymphohistiocytosis
Hemophagocytic syndrome
Multisystemic inflammation
Adult-onset hemophagocytosis
Uvea
Retina