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Perspective: A Novel Prognostic for Sickle Cell Disease

Sickle hemoglobin (α(2)β(S)(2)) polymerization drives disease pathophysiology in sickle cell anemia. Fetal hemoglobin (α(2)γ(2)) restricts disease severity by inhibiting the polymerization of sickle hemoglobin in a concentration-dependent manner. Clinical decision-making relies on diagnostic technol...

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Detalles Bibliográficos
Autores principales: Mozeleski, Brian M., Al-Rubaish, Abdullah, Al-Ali, Amein, Romero, Jose
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196691/
https://www.ncbi.nlm.nih.gov/pubmed/30787839
http://dx.doi.org/10.4103/sjmms.sjmms_107_18
Descripción
Sumario:Sickle hemoglobin (α(2)β(S)(2)) polymerization drives disease pathophysiology in sickle cell anemia. Fetal hemoglobin (α(2)γ(2)) 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.