背景:Yt系统由五种抗原组成:反Yta/Ytb和高流行抗原YTEG,YTLI,YTOT。我们调查了一名美国原住民(NA)女性的样本,该女性患有术后贫血和一种未识别的抗体,该抗体发展为严格的,心动过速,和低血压输注不相容的红细胞。
方法:血清学检测方法包括LISS,PEG,和IgG凝胶。试验红细胞用木瓜蛋白酶处理,胰蛋白酶,α-胰凝乳蛋白酶,2-氨基乙基异硫脲,和二硫苏糖醇。对稀有红细胞进行了测试,并进行了抑制研究。从WBC提取的DNA用于Sanger测序。
结果:初始测试显示在LISSIAT处与所有组细胞的强3-4+血浆反应性;自动控制是阴性的。除PNH-III外,许多罕见的红细胞均观察到阳性反应,缺乏GPI链接的DO,Yt,CROM,JMH,还有Emm.酶敏感性模式表明Yt特异性,和可溶性重组srYt中和反应性。ACHE测序显示YT*A/A基因型,但外显子2具有纯合变化,c.290A>G(p。Gln97Arg)。抗体反应性让人想起先前研究的无关NA男性。他的红细胞与她的血浆无反应。ACHE进行了相同的c.290G/G更改。
结论:发现两名无关NA患者对一种新的高流行Yt抗原抗体,指定YTGT(YT6),与临床上显著的输血反应有关。特异性的鉴定依赖于酶的敏感性,使用PNH-III红细胞,使用可溶性重组Yt进行中和,以及发现ACHE的新颖变化,c.290A>G(p。Gln97Arg),指定YT*01。-06.IVIG和类固醇用于减轻对输血的进一步反应。
BACKGROUND: The Yt system consists of five antigens: antithetical Yta /Ytb and the high-prevalence antigens YTEG, YTLI, and YTOT. We investigated a sample from a Native American (NA) female with post-operative anemia and an unidentified antibody who developed rigors, tachycardia, and hypotension on transfusion of incompatible RBCs.
METHODS: Serologic testing methods included LISS, PEG, and IgG gel. Test RBCs were treated with papain, trypsin, alpha-chymotrypsin, 2-amino-ethylisothiouronium, and dithiothreitol. Rare RBCs were tested, and inhibition studies were performed. DNA extracted from WBCs was used for Sanger sequencing.
RESULTS: Initial testing showed strong 3-4+ plasma reactivity with all panel cells at LISS IAT; auto control was negative. Positive reactions were observed with numerous rare RBCs except for PNH-III, which lack GPI-linked DO, Yt, CROM, JMH, and Emm. Enzyme sensitivity patterns suggest Yt specificity, and soluble recombinant srYt neutralized reactivity. ACHE sequencing revealed YT*A/A genotype but with a homozygous change in exon 2, c.290A>G (p.Gln97Arg). Antibody reactivity was reminiscent of that seen in an unrelated NA male investigated previously. His RBCs were nonreactive with her plasma. ACHE carried the same c.290G/G change.
CONCLUSIONS: Two unrelated NA patients were found to have an antibody to a new high-prevalence Yt antigen, designated YTGT (YT6), associated with a clinically significant transfusion reaction. Identification of the specificity relied on enzyme sensitivity, use of PNH-III RBCs, neutralization using soluble recombinant Yt, and the finding of a novel change in ACHE, c.290A>G (p.Gln97Arg), designated YT*01.-06. IVIG and steroids were used to mitigate further reactions to transfusion.