Article
The paradox of hemoglobin SC disease.
Blood reviews - 1 Sept 2003
Nagel Ronald L, Fabry Mary E, Steinberg Martin H
Abstract excerpt
Homozygous HbC gene results only in mild hemolytic anemia. In HbSC disease red cells contain equal levels of HbS and HbC. It is a paradox that HbSC exhibit a moderately severe phenotype in spite of being a mixture of HbS trait and HbC trait, neither of which has significant pathology. Why does the combination of these two Hbs result in a serious disease? The short answer is that HbC enhances, by dehydrating the...
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