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在一个南非家庭中,血红蛋白M-海德公园与多凝集红细胞相关。

Haemoglobin M-Hyde Park associated with polyagglutinable red blood cells in a South African family.

作者信息

Bird A R, Kent P, Moores P P, Elliott T

机构信息

Department of Haematology, Groote Schuur Hospital, University of Cape Town, South Africa.

出版信息

Br J Haematol. 1988 Apr;68(4):459-64. doi: 10.1111/j.1365-2141.1988.tb04236.x.

Abstract

Twelve of 35 members tested in a large ethnically-mixed South African family were found to have both haemoglobin M type Hyde Park and persistent polyagglutinable red blood cells. The characteristics of the polyagglutination have not been recorded previously. The cells of affected family members were not agglutinated by Arachis hypogea, Dolichos biflorus or Salvia sclarea, but were agglutinated weakly by Salvia horminum and BSII (GSII) and reacted strongly with Glycine soja and Sophora japonica lectins. BSI (GSI) lectin agglutinated the group A but not the group O cells. The N and MN cells were agglutinated more strongly than normal by Vicia graminea, other anti-N lectins and human anti-N but the M and MN cells reacted as expected with human anti-M. The name 'Hyde Park' is provisionally suggested for this type of polyagglutination, although it appears unlikely that the evidently complete association between the polyagglutination and the variant haemoglobin is the result of a single genetic mutation. More likely, the connection has a post-genetic origin, perhaps showing that bonds, possibly affected adversely by precocious senescence, normally occur between the haemoglobin and alpha-sialoglycoprotein molecules in red blood cells.

摘要

在一个种族混合的南非大家庭中接受检测的35名成员里,有12人被发现同时患有血红蛋白M海德公园型和持续性多凝集红细胞。此前尚未记录这种多凝集的特征。患病家庭成员的细胞不被落花生、双花扁豆或鼠尾草凝集,但被药用鼠尾草和BSII(GSII)弱凝集,并与野生大豆和槐凝集素发生强烈反应。BSI(GSI)凝集素凝集A组细胞,但不凝集O组细胞。蚕豆、其他抗N凝集素和人抗N对N和MN细胞的凝集作用比正常情况更强,但M和MN细胞与人抗M的反应符合预期。尽管这种多凝集与变异血红蛋白之间明显的完全关联不太可能是单一基因突变的结果,但暂时建议将这种多凝集类型命名为“海德公园型”。更有可能的是,这种关联有一个基因后起源,也许表明正常情况下红细胞中的血红蛋白和α-唾液酸糖蛋白分子之间存在的结合,可能受到早熟衰老的不利影响。

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