While Roth spots have been classically described in bacterial endocarditis, they may occur in a number of systemic conditions, including lymphoproliferative disorders.
Hemophagocytic lymphohistiocytosis is a rare disease with a range of ocular findings, and a study has revealed a case which also included the formation of Roth spots in the eyes. The patient was a 33-year-old African-American male with a history of human immunodeficiency virus (HIV) and hypertension who presented with fever, splenomegaly, and bilateral vision loss.
Multiple Roth spots were found bilaterally by ophthalmoscopy. The patient was found to have hypertriglyceridemia, elevated serum ferritin, pancytopenia, and elevated soluble interleukin 2 receptor levels.
A bone marrow biopsy confirmed hemophagocytic lymphohistiocytosis. Hemophagocytic lymphohistiocytosis is rare and can be a life-threatening, multisystem, inflammatory syndrome caused by overactive macrophages and lymphocytes.
Though ocular involvement in hemophagocytic lymphohistiocytosis is known, to our knowledge, this is the first case of Roth spots as the presenting ocular sign of hemophagocytic lymphohistiocytosis in the English-language ophthalmic literature. Clinicians should be aware of the unusual but characteristic signs, symptoms, and findings.
The patient had a history of HIV and hypertension on active antiretroviral therapy (HAART), admitted for fever and refractive pancytopenia, reported acute, painless, bilateral central vision loss.
Andrew G. Lee, MD
E: [email protected]
Dr. Lee, editor of The Neuro-Connection column, is affiliated with the Blanton Eye Institute, Houston Methodist Hospital. He did not indicate proprietary interest.
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