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一例血红蛋白广岛（β146 组氨酸突变为天冬氨酸）伴代偿性红细胞增多症和 HbA₁c 检测不出。

A case of hemoglobin Hiroshima (β146 histidine to aspartic acid) with compensatory erythremia and undetectable HbA₁c.

机构信息

Department of Internal Medicine, Teraoka Memorial Hospital, Fukuyama, Japan.

出版信息

Int J Hematol. 2012 Jun;95(6):697-701. doi: 10.1007/s12185-012-1066-1. Epub 2012 Apr 8.

DOI:10.1007/s12185-012-1066-1
PMID:22484594
Abstract

Hemoglobin (Hb) Hiroshima is an Hb variant that travels rapidly on electrophoresis and shows a fourfold increase in oxygen affinity and a decreased Bohr effect. We encountered a 40-year-old male patient with erythremia and an undetectable HbA(1c) level. The presence of an abnormal hemoglobin molecule was suggested by the results of high-performance liquid chromatography analysis. Subsequent gene analysis by direct sequencing confirmed Hb Hiroshima (β146 histidine → aspartic acid). Caution should be exercised when diagnosing erythremia.

摘要

血红蛋白（Hb）广岛变体在电泳时移动迅速，呈现出氧亲和力增加四倍和波尔效应降低的特点。我们遇到了一位 40 岁的男性红细胞增多症患者，HbA1c 水平无法检测。高效液相色谱分析结果提示存在异常血红蛋白分子。随后的直接测序基因分析证实了 Hb 广岛变体（β146 组氨酸→天冬氨酸）的存在。在诊断红细胞增多症时应谨慎。

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A case of hemoglobin Hiroshima (β146 histidine to aspartic acid) with compensatory erythremia and undetectable HbA₁c.一例血红蛋白广岛（β146 组氨酸突变为天冬氨酸）伴代偿性红细胞增多症和 HbA₁c 检测不出。
Int J Hematol. 2012 Jun;95(6):697-701. doi: 10.1007/s12185-012-1066-1. Epub 2012 Apr 8.
2
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Molecular basis and hematological features of hemoglobin variants in Southern Thailand.泰国南部血红蛋白变异体的分子基础和血液学特征。
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世界卫生组织真性红细胞增多症、原发性血小板增多症和原发性骨髓纤维化诊断标准修订提案及理由：一个特设国际专家小组的建议
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Int J Hematol. 2006 Aug;84(2):186-7. doi: 10.1532/IJH97.06108.
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Hemoglobin. 2005;29(2):91-106.
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Hemoglobin. 2003 May;27(2):117-21. doi: 10.1081/hem-120021546.
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Hemoglobin. 2002 Feb;26(1):1-5. doi: 10.1081/hem-120002934.
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Hb Tsukumi [beta117(G19)His-->Tyr]: a new hemoglobin variant found in a Japanese male.血红蛋白筑波[β117（G19）组氨酸→酪氨酸]：在一名日本男性中发现的一种新的血红蛋白变体。
Hemoglobin. 2000 May;24(2):117-23. doi: 10.3109/03630260009003430.
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Intern Med. 1999 Oct;38(10):800-3. doi: 10.2169/internalmedicine.38.800.
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