Home Print this page Email this page Users Online: 91
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
INVITED ARTICLE
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
USA
Login to access the Email id

DOI: 10.4103/sjmms.sjmms_107_18

Rights and Permissions

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.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed523    
    Printed59    
    Emailed0    
    PDF Downloaded148    
    Comments [Add]    

Recommend this journal