Perspective: A novel prognostic for sickle cell disease
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 eval...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2018-01-01
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| Series: | Saudi Journal of Medicine and Medical Sciences |
| Subjects: | |
| Online Access: | http://www.sjmms.net/article.asp?issn=1658-631X;year=2018;volume=6;issue=3;spage=133;epage=136;aulast=Mozeleski |
| _version_ | 1819080593118330880 |
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| author | Brian M Mozeleski Abdullah Al-Rubaish Amein Al-Ali Jose Romero |
| author_facet | Brian M Mozeleski Abdullah Al-Rubaish Amein Al-Ali Jose Romero |
| author_sort | Brian M Mozeleski |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-21T19:47:21Z |
| format | Article |
| id | doaj.art-6da46521932448a1bbd33c6d6d4362b0 |
| institution | Directory Open Access Journal |
| issn | 1658-631X |
| language | English |
| last_indexed | 2024-12-21T19:47:21Z |
| publishDate | 2018-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Saudi Journal of Medicine and Medical Sciences |
| spelling | doaj.art-6da46521932448a1bbd33c6d6d4362b02022-12-21T18:52:17ZengWolters Kluwer Medknow PublicationsSaudi Journal of Medicine and Medical Sciences1658-631X2018-01-016313313610.4103/sjmms.sjmms_107_18Perspective: A novel prognostic for sickle cell diseaseBrian M MozeleskiAbdullah Al-RubaishAmein Al-AliJose RomeroSickle 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.http://www.sjmms.net/article.asp?issn=1658-631X;year=2018;volume=6;issue=3;spage=133;epage=136;aulast=MozeleskiFetal hemoglobinsickle cell diseasesickle hemoglobin |
| spellingShingle | Brian M Mozeleski Abdullah Al-Rubaish Amein Al-Ali Jose Romero Perspective: A novel prognostic for sickle cell disease Saudi Journal of Medicine and Medical Sciences Fetal hemoglobin sickle cell disease sickle hemoglobin |
| 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 | Fetal hemoglobin sickle cell disease sickle hemoglobin |
| url | http://www.sjmms.net/article.asp?issn=1658-631X;year=2018;volume=6;issue=3;spage=133;epage=136;aulast=Mozeleski |
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