Krügner F, Zaccariotto T R, Rosim E T, Costa F F, Grotto H Z W, Sonati M F
Department of Clinical Pathology, School of Medical Sciences, State University of Campinas-UNICAMP, Campinas, State of São Paulo, Brazil.
Am J Hematol. 2006 Jan;81(1):68-70. doi: 10.1002/ajh.20487.
Although it is almost certain that alpha(+)-thalassemia protects against malaria, the mechanisms for that are still unknown. It has been suggested that an increased number of young circulating red blood cells in alpha(+)-thalassemic children, as a result of some degree of ineffective erythropoiesis, could be related to the high frequencies of the alpha(+)-thalassemic allele in malaria endemic areas. Reticulocyte evaluation in this condition, however, has been poorly performed so far. Our objective was to determine the reticulocyte number and maturation degree, in addition to the soluble transferrin receptor and serum erythropoietin levels, in alpha(+)-thalassemia heterozygotes, comparing them with normal alpha-genotype controls. One hundred twenty-one alpha(+)-thalassemia carriers (-alpha(3.7)/alphaalpha) and 249 controls (alphaalpha/alphaalpha), all of them with normal serum ferritin levels, were subclassified according to age (1-5, 6-10, 11-15, 16-20, and over 20 years old). Reticulocyte analyzes were carried out by flow cytometry and sTfR and s-Epo levels determined by immunonephelometry and chemiluminescence, respectively. The comparisons did not show any significant difference between thalassemics and controls regarding the reticulocyte parameters [percentages and absolute values, P = 0.2643 and 0.5421; high, medium, and low maturation degree, P = 0.2579, 0.2196, and 0.4192; RET maturity index (RMI), P = 0.2471, respectively], as well as the s-Epo levels (P = 0.5711). The sTfR concentrations were higher in the thalassemic group (P = 0.0001), but statistical significance was due only to the 1-5 and over 20 subgroups (P = 0.0082 and 0.0436, respectively). The results found here are compatible with a compensated erythropoiesis and do not confirm the hypothesis mentioned above.
尽管几乎可以确定α(+)-地中海贫血能预防疟疾,但其机制仍不清楚。有人提出,由于某种程度的无效红细胞生成,α(+)-地中海贫血儿童循环中的年轻红细胞数量增加,这可能与疟疾流行地区α(+)-地中海贫血等位基因的高频率有关。然而,到目前为止,在这种情况下对网织红细胞的评估做得很少。我们的目标是测定α(+)-地中海贫血杂合子中网织红细胞数量和成熟度,以及可溶性转铁蛋白受体和血清促红细胞生成素水平,并将其与正常α基因型对照进行比较。121名α(+)-地中海贫血携带者(-α(3.7)/αα)和249名对照(αα/αα),他们的血清铁蛋白水平均正常,根据年龄(1 - 5岁、6 - 10岁、11 - 15岁、16 - 20岁和20岁以上)进行亚分类。通过流式细胞术进行网织红细胞分析,分别通过免疫比浊法和化学发光法测定可溶性转铁蛋白受体(sTfR)和血清促红细胞生成素(s-Epo)水平。比较结果显示,地中海贫血患者和对照组在网织红细胞参数[百分比和绝对值,P = 0.2643和0.5421;高、中、低成熟度,P = 0.2579、0.2196和0.4192;网织红细胞成熟指数(RMI),P = 0.2471]以及s-Epo水平(P = 0.5711)方面均无显著差异。地中海贫血组的sTfR浓度较高(P = 0.0001),但统计学显著性仅归因于1 - 5岁和20岁以上亚组(分别为P = 0.0082和0.0436)。此处发现的结果与代偿性红细胞生成相符,并未证实上述假设。