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At(a-) 表型:一个家族的描述以及在一名带有抗 - Ata 的先证者中 At(a+) 红细胞的存活减少情况。

At(a-) phenotype: description of a family and reduced survival of At(a+) red cells in a proposita with anti-Ata.

作者信息

Sweeney J D, Holme S, McCall L, Huett D, Storry J, Reid M

机构信息

American Red Cross Blood Services, Mid-Atlantic Region, Norfolk, Virginia.

出版信息

Transfusion. 1995 Jan;35(1):63-7. doi: 10.1046/j.1537-2995.1995.35195090666.x.

DOI:10.1046/j.1537-2995.1995.35195090666.x
PMID:7998074
Abstract

BACKGROUND

There is a paucity of data on the August (At) blood group antigen and clinical significance of anti-Ata.

STUDY DESIGN AND METHODS

A proposita with the At(a-) phenotype was identified by the finding of anti-Ata in the cord blood eluate of her fifth live infant. Family members were studied, and a small aliquot of 51Cr-labeled At(a+) red cells was transfused to determine survival.

RESULTS

There was no evidence of hemolytic disease of the newborn, as determined by the normal hemoglobin and bilirubin and normal clinical conditions. Six of seven siblings were tested, and two At(a-) female siblings were identified. In contrast to the proposita, neither sister had detectable anti-Ata in her serum, although each has had only one pregnancy. A monocyte monolayer assay performed on serum from the proposita gave a result of 20-percent hemolysis (normal, <3%), which is consistent with a clinically significant antibody. Transfusion of a small volume of allogeneic red cells that were phenotypically matched with the proposita, except for Ata, resulted in a 1-hour survival of 95 percent, but a 24-hour survival of only 18 percent, of the transfused cells. The survival pattern was exponential, which is characteristic of a non-complement-binding IgG antibody.

CONCLUSION

Despite the absence of hemolytic disease of the newborn, this example of anti-Ata would appear to be a clinically significant antibody for the purposes of transfusion practice. Therefore, approaches to the management of clinical situations in which transfusion is required or likely should focus on the availability of autologous cells or frozen allogeneic At(a-) red cells.

摘要

背景

关于奥古斯特(At)血型抗原及抗-Ata的临床意义的数据匮乏。

研究设计与方法

通过在其第五个存活婴儿的脐血洗脱液中发现抗-Ata,确定了一名具有At(a-)表型的先证者。对家庭成员进行了研究,并输注了一小份51Cr标记的At(a+)红细胞以确定其存活情况。

结果

根据正常的血红蛋白、胆红素水平及正常的临床状况,未发现新生儿溶血病的证据。对七个兄弟姐妹中的六个进行了检测,确定了两个At(a-)女性兄弟姐妹。与先证者不同,尽管每个姐妹仅怀孕一次,但她们的血清中均未检测到抗-Ata。对先证者血清进行的单核细胞单层试验结果为20%溶血(正常,<3%),这与具有临床意义的抗体相符。输注少量除Ata外与先证者表型匹配的异体红细胞,输注细胞的1小时存活率为95%,但24小时存活率仅为18%。存活模式呈指数形式,这是非补体结合IgG抗体的特征。

结论

尽管未出现新生儿溶血病,但就输血实践而言,这个抗-Ata的例子似乎是一种具有临床意义的抗体。因此,对于需要或可能需要输血的临床情况的处理方法应侧重于自体细胞或冷冻异体At(a-)红细胞的可用性。

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