Immune parameter analysis of children with sickle cell disease on hydroxycarbamide or chronic transfusion therapy

• Type of Material: Article
• Authors: Robert S. Nickel, Emory University; Ifeyinwa Osunkwo, Carolinas Medical Centre; Aneesah Garrett, Emory University; Jennifer Robertson, Emory University; David Archer, Emory University; Daniel E.L. Promislow, University of Washington; John Horan, Emory University; Jeanne Hendrickson, Emory University; Leslie Kean, Emory University
• Language: English
• Date: 2015-05-01
• Publisher: Wiley
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2015 John Wiley & Sons Ltd.
• Final Published Version (URL): http://dx.doi.org/10.1111/bjh.13326
• Title of Journal or Parent Work: British Journal of Haematology
• ISSN: 0007-1048
• Volume: 169
• Issue: 4
• Start Page: 574
• End Page: 583
• Grant/Funding Information: This work was supported by a Children’s Healthcare of Atlanta Centre for Transplantation and Immune-Mediated Disorders Pilot Grant (IO, JTH, JEH, LSK), the Hudgens Family Foundation and the National Centre for Advancing Translational Sciences of the NIH under Award Number UL1TR000454 (RSN).
• Keywords: Life Sciences & Biomedicine; HYDROXYUREA; hydroxycarbamide; IRON OVERLOAD; ANEMIA; BLOOD-TRANSFUSIONS; sickle cell disease; ACTIVATED MONOCYTES; chronic transfusion; immunophenotype; BABY HUG; VASCULAR ENDOTHELIUM; immune phenotype; TRANSPLANTATION; NATURAL-KILLER ACTIVITY; Hematology
• Research Categories: Health Sciences, Pathology; Health Sciences, Immunology

Abstract

Sickle cell disease (SCD) is increasingly appreciated as an inflammatory condition associated with alterations in immune phenotype and function. In this cross-sectional study we performed a multiparameter analysis of 18 immune markers in 114 paediatric SCD patients divided by treatment group [those receiving hydroxycrabamide (HC, previously termed hydroxyurea), chronic transfusion (CT), or no disease-modifying therapy] and 29 age-matched African American healthy controls. We found global elevation of most immune cell counts in SCD patients receiving no disease-modifying therapy at steady state. Despite the decrease in percentage of haemoglobin S associated with CT therapy, the abnormal cellular immune phenotype persisted in patients on CT. In contrast, in both univariate and multivariate analysis, treatment with HC was associated with normalization of the vast majority of leucocyte populations. This study provides additional support for HC treatment in SCD, as it appears that HC decreases the abnormally elevated immune cell counts in patients with SCD.