Barton James C, Wiener Howard W, Acton Ronald T, Go Rodney Cp
Southern Iron Disorders Center, Birmingham, Alabama, USA.
Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.
BMC Blood Disord. 2005 Jul 25;5:5. doi: 10.1186/1471-2326-5-5.
It has been reported that some persons with hemochromatosis have low total blood lymphocyte counts, but the reason for this is unknown.
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.
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-A01, -B08, and -B14 alleles, and the A01-B08 haplotype. Presence of the A01 allele, B08 allele, or A01-B08 haplotype were associated with a lower total blood lymphocyte count, whereas presence of the B14 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 A01-B08 haplotype.
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-A01 allele or the -B08 allele was also associated with significantly lower total blood lymphocyte counts, whereas presence of the -B14 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-A01-B*08 haplotype than in probands without this haplotype.
据报道，一些血色素沉着症患者的全血淋巴细胞计数较低，但其原因尚不清楚。
我们使用自动血细胞计数器对146名经医疗诊断为HFE C282Y纯合子的血色素沉着症先证者（88名男性，58名女性）进行了全血淋巴细胞计数测量。使用以下变量对全血淋巴细胞计数进行单因素和多因素分析：性别；年龄、诊断时的转铁蛋白饱和度和血清铁蛋白浓度；为实现铁耗竭通过放血去除的血量；以及人类白细胞抗原（HLA）-A和-B等位基因及单倍型。
男性诊断时的平均年龄为49±14岁（范围18 - 80岁），女性为50±13岁（范围22 - 88岁）。全血淋巴细胞计数与性别、年龄、诊断时的转铁蛋白饱和度和血清铁蛋白浓度，以及为实现铁耗竭通过放血去除的血量之间的相关性在0.05水平上不显著。单因素分析显示全血淋巴细胞计数与HLA-A01、-B08和-B14等位基因以及A01-B08单倍型的存在之间存在显著关联。A01等位基因、B08等位基因或A01-B08单倍型的存在与较低的全血淋巴细胞计数相关，而B14等位基因的存在与较高的全血淋巴细胞计数相关。全血淋巴细胞计数与为实现铁耗竭的放血单位数、血清铁蛋白浓度以及A01-B08单倍型的存在呈负相关。
我们得出结论，在HFE C282Y纯合子的血色素沉着症先证者中，全血淋巴细胞计数与铁过载严重程度之间存在显著的负相关关系。HLA-A01等位基因或-B08等位基因的存在也与显著较低的全血淋巴细胞计数相关，而-B14等位基因的存在与显著较高的全血淋巴细胞计数相关。在单因素和多因素分析中，具有HLA-A01-B*08单倍型的先证者的全血淋巴细胞计数显著低于没有该单倍型的先证者。
