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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload

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Perspective: A Novel Prognostic for Sickle Cell Disease

Brian M Mozeleski1, Abdullah Al-Rubaish2, Amein Al-Ali3

  • 1MyoSyntax Corporation, Worcester, Massachusetts, USA.

Saudi Journal of Medicine & Medical Sciences
|February 22, 2019
PubMed

Sickle hemoglobin (α2βS 2) polymerization drives disease pathophysiology in sickle cell anemia. Fetal hemoglobin (α2γ2) restricts disease severity by inhibiting the polymerization of sickle hemoglobin in a concentration-dependent manner. Clinical decision-making relies on diagnostic technologies evaluating fetal hemoglobin as mean percent or mean quantity in blood. Limitation of this approach is exemplified by patients with significant high fetal hemoglobin levels and severe disease, suggesting that fetal hemoglobin is unevenly distributed across F-cells. Therefore, determination of fetal hemoglobin/F-cell would provide a new paradigm for ascertaining prognosis and response to fetal hemoglobin-inducing agents. Measurement of fetal hemoglobin/F-cell, ultimately adapted to widespread standardized analytical use, is a promising fetal hemoglobin-related prognostic approach to monitor the severity of sickle cell disease and the best "phenotype" to follow when developing new candidate fetal hemoglobin inducers or titrating hydroxyurea in treated sickle cell patients.

Keywords:
Fetal hemoglobinsickle cell diseasesickle hemoglobin

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