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钴胺素缺乏所致巨幼细胞贫血中的血清转铁蛋白受体

Serum transferrin receptor in the megaloblastic anemia of cobalamin deficiency.

作者信息

Carmel R, Skikne B S

机构信息

Department of Medicine, University of Southern California School of Medicine, Los Angeles 90033.

出版信息

Eur J Haematol. 1992 Nov;49(5):246-50. doi: 10.1111/j.1600-0609.1992.tb00056.x.

DOI:10.1111/j.1600-0609.1992.tb00056.x
PMID:1473586
Abstract

In order to further study the relation between transferrin receptor and erythropoiesis we examined serum receptor levels in megaloblastic anemia, which is the classic example of ineffective erythropoiesis. We studied 33 patients with unequivocal cobalamin deficiency, only 22 of whom were anemic. High serum transferrin receptor levels were found in 12 patients, all of whom were anemic and had high lactate dehydrogenase (LDH) levels; in contrast, only 10 of the 21 patients with normal receptor levels were anemic. Receptor correlated most strongly with LDH (r = 0.573, p < 0.001) and, inversely, with hemoglobin values (r = -0.560, p < 0.001); it also correlated with ferritin and total bilirubin levels, but not with cobalamin, MCV or erythropoietin. No association was found with the hemolytic component of megaloblastic anemia, represented indirectly by haptoglobin levels. Changes induced by cobalamin therapy were also examined in 13 patients. Transferrin receptors rose in all 6 patients who initially had high levels and in 2 of 3 patients who had borderline levels, but not in the 4 patients with initially normal levels. The receptor levels began to rise within 1-3 days, peaked at about 2 weeks and returned to normal at about the 5th wk. The findings indicate that serum transferrin receptor levels reflect the severity of the megaloblastic anemia. The elevated receptor levels rise further with cobalamin therapy, however, as effective erythropoiesis replaces ineffective erythropoiesis, and these persist until the increased erythropoiesis returns to normal.

摘要

为了进一步研究转铁蛋白受体与红细胞生成之间的关系,我们检测了巨幼细胞贫血患者的血清受体水平,巨幼细胞贫血是无效红细胞生成的经典例子。我们研究了33例明确存在钴胺素缺乏的患者,其中只有22例贫血。12例患者血清转铁蛋白受体水平较高,所有这些患者均贫血且乳酸脱氢酶(LDH)水平较高;相比之下,21例受体水平正常的患者中只有10例贫血。受体与LDH相关性最强(r = 0.573,p < 0.001),与血红蛋白值呈负相关(r = -0.560,p < 0.001);它还与铁蛋白和总胆红素水平相关,但与钴胺素、平均红细胞体积(MCV)或促红细胞生成素无关。未发现与以触珠蛋白水平间接代表的巨幼细胞贫血的溶血成分有关联。我们还对13例患者进行了钴胺素治疗引起的变化检测。最初受体水平较高的所有6例患者以及3例临界水平患者中的2例,转铁蛋白受体水平升高,但最初水平正常的4例患者未升高。受体水平在1 - 3天内开始升高,约2周时达到峰值,约第5周时恢复正常。这些发现表明血清转铁蛋白受体水平反映了巨幼细胞贫血的严重程度。然而,随着有效的红细胞生成取代无效的红细胞生成,钴胺素治疗后升高的受体水平会进一步升高,并且这些水平会持续到增加的红细胞生成恢复正常。

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