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Protection from sickle cell retinopathy is associated with elevated HbF levels and hydroxycarbamide use in children

Jeremie H Estepp1, Matthew P Smeltzer, Winfred C Wang

  • 1Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN, USA. jeremie.estepp@stjude.org

British Journal of Haematology
|February 7, 2013
PubMed

Insights

Elevated fetal hemoglobin (HbF) protects against sickle cell retinopathy in children. Lower HbF levels significantly increase retinopathy risk, suggesting hydroxycarbamide therapy may prevent this complication.

Area of Science:

  • Hematology
  • Ophthalmology
  • Pediatrics

Background:

  • Sickle cell anemia (HbSS) is a genetic blood disorder with various complications.
  • Elevated fetal hemoglobin (HbF) is known to ameliorate some sickle cell disease manifestations.
  • The effect of HbF on sickle cell retinopathy remains unclear.

Purpose of the Study:

  • To investigate the association between fetal hemoglobin levels and the development of retinopathy in children with sickle cell anemia.
  • To determine if elevated HbF has a protective effect against retinopathy in this population.

Main Methods:

  • Retrospective analysis of 123 children with HbSS.
  • Assessment of retinopathy prevalence and correlation with HbF levels.
  • Evaluation of HbF levels in children treated with hydroxycarbamide.

Main Results:

  • 10.6% of children with HbSS developed retinopathy.
  • Children with HbF <15% had a 7.1-fold higher odds of developing retinopathy, independent of hydroxycarbamide treatment.
  • In hydroxycarbamide-treated children, those with retinopathy had significantly lower HbF levels (9% vs. 16%, P = 0.005).

Conclusions:

  • Elevated fetal hemoglobin (HbF) demonstrates a protective effect against retinopathy in children with sickle cell anemia (HbSS).
  • Data suggests that hydroxycarbamide therapy, by inducing HbF, may serve as a preventative strategy for retinopathy in these children.

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