title: Immune parameter analysis of children with sickle cell disease on hydroxycarbamide or chronic transfusion therapy.
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.
authors.affiliation: Aflac Cancer and Blood Disorders Centre, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA; Centre for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine, Emory University, Atlanta, GA, USA.
authors.forename: Robert S
authors.initials: RS
authors.lastname: Nickel
authors.forename: Ifeyinwa
authors.initials: I
authors.lastname: Osunkwo
authors.forename: Aneesah
authors.initials: A
authors.lastname: Garrett
authors.forename: Jennifer
authors.initials: J
authors.lastname: Robertson
authors.forename: David R
authors.initials: DR
authors.lastname: Archer
authors.forename: Daniel E L
authors.initials: DE
authors.lastname: Promislow
authors.forename: John T
authors.initials: JT
authors.lastname: Horan
authors.forename: Jeanne E
authors.initials: JE
authors.lastname: Hendrickson
authors.forename: Leslie S
authors.initials: LS
authors.lastname: Kean
mesh_terms: D000293:Adolescent; D001741:Black or African American; D000755:Anemia, Sickle Cell / Q000097:blood / Q000276:immunology* / Q000628:therapy*; D000986:Antisickling Agents / Q000008:administration & dosage*; D001803:Blood Transfusion; D002648:Child; D005500:Follow-Up Studies; D006801:Humans; D006918:Hydroxyurea / Q000008:administration & dosage*; D007958:Leukocyte Count; D007962:Leukocytes / Q000276:immunology*
chemical_list: D000986:Antisickling Agents; D006918:Hydroxyurea
keywords: chronic transfusion; hydroxycarbamide; immune phenotype; immunophenotype; sickle cell disease