Abstract
Background: Deferoxamine retinopathy is the informally designated term used to describe a characteristic pattern of outer retinal degeneration in iron-overloaded chronic anemia patients who are treated with deferoxamine. We hypothesize that insufficiently treated iron overloading and not only deferoxamine is the cause of the retinal degeneration. Our case report is based on exposure histories of two anemia patients and literature review. Case presentation: Both anemia patients presented with bilateral visual loss secondary to photoreceptor and retinal pigment epithelium degeneration. Chart review showed that visual loss came after a year-long slow, and rather monotonous rise in plasma ferritin concentrations, with no obvious relation to iron chelator exposure. In one patient, the onset of symptomatic visual loss came after a bout of fever followed by two additional febrile episodes, all accompanied by plasma ferritin spikes. Adjustment of iron chelation therapy did not improve visual function. Experimental studies clearly show that both systemic and intraocular exposure to iron ions can induce retinal degeneration. Conclusion: The available evidence indicates that retinal degeneration in chronic anemia patients treated by deferoxamine is cause by insufficient iron chelation, not by deferoxamine. The actual role of iron chelating agents may be to promote a long enough survival to allow the slow development of retinal siderosis.
Originalsprog | Engelsk |
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Artikelnummer | 277 |
Tidsskrift | BMC Ophthalmology |
Vol/bind | 21 |
Antal sider | 7 |
ISSN | 1471-2415 |
DOI | |
Status | Udgivet - 2021 |
Bibliografisk note
Funding Information:The study was supported by the European Union under H2020-EU.2.1.1. - ID 780989 (MERLIN); Rigshospitalet(grant E-23334-02); P. Carl Petersens Fond (grant 19102); Aase og Ejnar Danielsens Fond (grant 18-10-0698); Helsefonden (grant 19-B-0063); Beckett Fonden (grant 19-2-3490); and Einar Willumsens Mindelegat (grant 500028). None of the above-mentioned sources has had any role in the design, analysis or interpretation of data and in writing the manuscript.
Publisher Copyright:
© 2021, The Author(s).