ArticleOcular oncology and pathology2017
Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases.
Article in Ocular oncology and pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed.
- Corneal perforation in the setting of NK/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis.American journal of ophthalmology case reports · 2026Article
- Multimodal Imaging Features of Retinal Lesions in Hemophagocytic Lymphohistiocytosis: A Clinical Analysis.Clinical ophthalmology (Auckland, N.Z.) · 2025Article
- A Case of the Initial Presentation of Hemophagocytic Lymphohistiocytosis as Acute Unilateral Vision Loss.Cureus · 2024Article
- Clinical approach to a child with hemophagocytic lymphohistiocytosis and bilateral optic nerve head infiltration: A case report and brief literature review.Clinical case reports · 2023Article
- Secondary Hemophagocytic Lymphohistiocytosis Causing Hemorrhagic Encephalomyelitis and Spinal Subarachnoid Hemorrhage: A Case Report.The Neurohospitalist · 2022Article
- Two Cases of Hemophagocytic Lymphohistiocytosis Associated with Disseminated Histoplasmosis Presented with Transient Pancytopenia.Case reports in medicine · 2022Article
- Diplopia and Vision Loss Associated With Presumed Systemic Lymphohistiocytic Disease: A Case Report.Clinical medicine insights. Case reports · 2022Article
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Authors and funding
3 authors.
Funding
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Abstract
BACKGROUND/
aimsHemophagocytic lymphohistiocytosis (HLH) is an infrequent inflammatory multisystemic syndrome. Only rare cases with ophthalmic involvement describing their pathologic features have been previously reported.
methodsWe report 3 cases of adult-onset HLH with bilateral ocular involvement and describe their clinicopathologic features.
resultsThree 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.
conclusionHLH 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.
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