Email updates

Keep up to date with the latest news and content from BMC Hematology and BioMed Central.

Open Access Highly Accessed Research article

Total blood lymphocyte counts in hemochromatosis probands with HFE C282Y homozygosity: relationship to severity of iron overload and HLA-A and -B alleles and haplotypes

James C Barton12, Howard W Wiener3, Ronald T Acton234* and Rodney CP Go3

  • * Corresponding author: Ronald T Acton acton@uab.edu

  • † Equal contributors

Author Affiliations

1 Southern Iron Disorders Center, Birmingham, Alabama, USA

2 Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA

3 Department of Epidemiology and International Health, University of Alabama at Birmingham, Birmingham, Alabama, USA

4 Immunogenetics Program, Department of Microbiology, University of Alabama at Birmingham, Birmingham, Alabama, USA

For all author emails, please log on.

BMC Blood Disorders 2005, 5:5  doi:10.1186/1471-2326-5-5

Published: 25 July 2005

Abstract

Background

It has been reported that some persons with hemochromatosis have low total blood lymphocyte counts, but the reason for this is unknown.

Methods

We measured total blood lymphocyte counts using an automated blood cell counter in 146 hemochromatosis probands (88 men, 58 women) with HFE C282Y homozygosity who were diagnosed in medical care. Univariate and multivariate analyses of total blood lymphocyte counts were evaluated using these variables: sex; age, transferrin saturation, and serum ferritin concentration at diagnosis; units of blood removed by phlebotomy to achieve iron depletion; and human leukocyte antigen (HLA)-A and -B alleles and haplotypes.

Results

The mean age at diagnosis was 49 ± 14 years (range 18 – 80 years) in men and 50 ± 13 years (range 22 – 88 years) in women. The correlations of total blood lymphocyte counts with sex, age, transferrin saturation, and serum ferritin concentration at diagnosis, and units of blood removed by phlebotomy to achieve iron depletion were not significant at the 0.05 level. Univariate analyses revealed significant associations between total blood lymphocyte counts and presence of the HLA-A*01, -B*08, and -B*14 alleles, and the A*01-B*08 haplotype. Presence of the A*01 allele, B*08 allele, or A*01-B*08 haplotype were associated with a lower total blood lymphocyte count, whereas presence of the B*14 allele was associated with a greater total blood lymphocyte count. There was an inverse association of total blood lymphocyte count with units of phlebotomy to achieve iron depletion, serum ferritin concentration, and with presence of the A*01-B*08 haplotype.

Conclusion

We conclude that there is a significant inverse relationship of total blood lymphocyte counts and severity of iron overload in hemochromatosis probands with HFE C282Y homozygosity. The presence of the HLA-A*01 allele or the -B*08 allele was also associated with significantly lower total blood lymphocyte counts, whereas presence of the -B*14 allele was associated with significantly higher total blood lymphocyte counts. In univariate and multivariate analyses, total blood lymphocyte counts were significantly lower in probands with the HLA-A*01-B*08 haplotype than in probands without this haplotype.