Association between plasma free haem and incidence of vaso-occlusive episodes and acute chest syndrome in children with sickle cell disease

• Type of Material: Article
• Authors: Olufolake Adisa, Emory University; Yijuan Hu, Emory University; Samit Ghosh, Emory University; Doreen Aryee, Emory University; Ifeyinwa Osunkwo, Emory University; Solomon F. Ofori-Acquah, Emory University
• Language: English
• Date: 2013-09-01
• Publisher: Wiley: 12 months
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2013 John Wiley & Sons.
• Final Published Version (URL): http://dx.doi.org/10.1111/bjh.12445
• Title of Journal or Parent Work: British Journal of Haematology
• ISSN: 0007-1048
• Volume: 162
• Issue: 5
• Start Page: 702
• End Page: 705
• Grant/Funding Information: This work was supported by the National Heart, Lung and Blood Institute (NHLBI) (Award Number: K02HL088026, R01HL106192); and a PHORG grant from the Aflac Cancer and Blood Disorders Center.
• Keywords: ANEMIA; acute chest syndrome; PULMONARY-HYPERTENSION; vaso-occlusive pain crisis; Science & Technology; Life Sciences & Biomedicine; DEATH; OXYGENASE-1; HEMOGLOBIN; sickle cell disease; Hematology; RISK; extracellular haem
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

We tested the hypothesis that extracellular haem is linked to the incidence of acute complications of sickle cell disease (SCD). Using multivariable regression analysis, higher plasma free haem, but not total plasma haem, was associated with increased odds of vaso-occlusive crisis (VOC) [P = 0·028, odds ratio (OR); 2·05, 95% Confidence Interval (CI) = 1·08-3·89] and acute chest syndrome (ACS) [P = 0·016, OR; 2·56, CI = 1·19, 5·47], after adjusting for age and gender in children with SCD. These findings suggest that haem and factors that influence its concentration in plasma may be informative of the risk of VOC and ACS in SCD patients.