Cargando…

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...

Descripción completa

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
_version_ 1783364606135107584
author Mozeleski, Brian M.
Al-Rubaish, Abdullah
Al-Ali, Amein
Romero, Jose
author_facet Mozeleski, Brian M.
Al-Rubaish, Abdullah
Al-Ali, Amein
Romero, Jose
author_sort Mozeleski, Brian M.
collection PubMed
description 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.
format Online
Article
Text
id pubmed-6196691
institution National Center for Biotechnology Information
language English
publishDate 2018
publisher Medknow Publications & Media Pvt Ltd
record_format MEDLINE/PubMed
spelling pubmed-61966912019-02-20 Perspective: A Novel Prognostic for Sickle Cell Disease Mozeleski, Brian M. Al-Rubaish, Abdullah Al-Ali, Amein Romero, Jose Saudi J Med Med Sci Invited Article 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. Medknow Publications & Media Pvt Ltd 2018 2018-08-14 /pmc/articles/PMC6196691/ /pubmed/30787839 http://dx.doi.org/10.4103/sjmms.sjmms_107_18 Text en Copyright: © 2018 Saudi Journal of Medicine & Medical Sciences http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Invited Article
Mozeleski, Brian M.
Al-Rubaish, Abdullah
Al-Ali, Amein
Romero, Jose
Perspective: A Novel Prognostic for Sickle Cell Disease
title Perspective: A Novel Prognostic for Sickle Cell Disease
title_full Perspective: A Novel Prognostic for Sickle Cell Disease
title_fullStr Perspective: A Novel Prognostic for Sickle Cell Disease
title_full_unstemmed Perspective: A Novel Prognostic for Sickle Cell Disease
title_short Perspective: A Novel Prognostic for Sickle Cell Disease
title_sort perspective: a novel prognostic for sickle cell disease
topic Invited Article
url 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
work_keys_str_mv AT mozeleskibrianm perspectiveanovelprognosticforsicklecelldisease
AT alrubaishabdullah perspectiveanovelprognosticforsicklecelldisease
AT alaliamein perspectiveanovelprognosticforsicklecelldisease
AT romerojose perspectiveanovelprognosticforsicklecelldisease