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Year : 2018  |  Volume : 6  |  Issue : 3  |  Page : 133-136

Perspective: A novel prognostic for sickle cell disease

1 MyoSyntax Corporation, Worcester, Massachusetts, USA
2 Department of Internal Medicine, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Kingdom of Saudi Arabia
3 Department of Biochemistry, College of Medicine, Imam Abdulrahman Bin Faisal University, Dammam, Kingdom of Saudi Arabia
4 Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA

Correspondence Address:
Brian M Mozeleski
MyoSyntax Corporation, 60 Prescott St, Worcester, MA 01605
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DOI: 10.4103/sjmms.sjmms_107_18

PMID: 30787839

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Sickle hemoglobin (α2βS2) 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.

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