Sickle Retinopathy in a Person with Hemoglobin S/New York Disease

A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease. This recently reported form of sickle cell disease was not pr...

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Bibliographic Details
Main Authors: Donovan Calder, Maryse Etienne-Julan, Marc Romana, Naomi Watkins, Jennifer M. Knight-Madden
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Genetics
Online Access:http://dx.doi.org/10.1155/2012/136582
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Summary:A patient who presented with sickle retinopathy and hemoglobin electrophoresis results compatible with sickle cell trait was found, on further investigation, to be a compound heterozygote with hemoglobin S and hemoglobin New York disease. This recently reported form of sickle cell disease was not previously known to cause retinopathy and surprisingly was observed in a non-Asian individual. The ophthalmological findings, the laboratory diagnosis, and possible pathophysiology of this disorder are discussed. Persons diagnosed with sickle cell trait who present with symptoms of sickle cell disease may benefit from specific screening for this variant.
ISSN:2090-6544
2090-6552