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[弱B血型B3、Bx、Bel的拟用实用分类]

[Proposed practical classification of weak B phenotypes B 3, Bx, B el].

作者信息

Salmon C

出版信息

Rev Fr Transfus Immunohematol. 1976 Mar;19(1):89-104. doi: 10.1016/s0338-4535(76)80090-6.

Abstract

Although the first weak B phenotypes have been observed some thirty years ago, very few comparative studies have been done until now. In this work, different samples were analysed, using immunogenetic methods, thermodynamics, agglutination kinetics and agglutination profiles. Almost hundred weak B samples were tested, belonging to twenty nine families including cis AB but exclusing acquired B and Bh. Using these methods it could be evidenced that the reactive structures differ from one family to another, but are strictly the same within one family. Consequently, each sample should have been called from the family mane; from a practical point of view, it was but necessary to agree on a simple classification. The simplest criteria were looked for such as agglutination velocity on a tile, agglutination picture, presence or absence of anti-B in serum... Three groups were then distinguished: 1) B3, for which the agglutination velocity is high (less than or equal to 30 inches), giving a typical mixed field agglutination pattern; there is no anti-B in the serum; 2) Bx, for which the agglutination velocity is low (larger than or equal to 30 inches), giving generally a weak agglutination pattern; there is a weak anti-B in serum; 3) Bel, for which there is no agglutination at all; the presence of B antigen on the membrane being evidenced by a fixation-elution test; in these three conditions genetic studies proved the phenotype being due to an allele at the ABO locus, the Bel class is the most "discutable"; when an important secretion of B substance can be evidenced in saliva, and the phenotype is proved to be inherited as a dominant character, the appellation Bm can be proposed. On the contrary, when genetics suggest the presence of a modifying gene, the Bel mod or By appellation can be used, according as the type of modification is dominant or recessive. Only one sample appeared to be unclassable, for which the fixation-elution test is positive, but only 5 to 10% of the red cells are agglutinated, giving a dual population pattern; the galactosyl-transferase activity seems to be normal, as well as the--delta H (20.000 cal/mol.). The B weak appellation appears to be the most appropriate for this given sample. This case expected, the proposed practical classification appears to fit well the studied phenotypes. As the various studies on weak B phenotypes, reported in the literature have not been compared within a same laboratory, using the same technics, it appears illusive to apply the proposed classification to the different reported samples. Several already published samples form a part of the present study, but their appellation do not correspond to the previous one; stricklingly, only few B3 (new appellation) have been described in the literature, which let one think that they might be undetected using classical grouping tests, and thus considered as normal B. Of course, there is not an absolute correspondance between the criteria defining A3, Ax, Ael phenotypes and those proposed here for B3, Bx, Bel...

摘要

尽管早在约三十年前就已观察到首例弱B血型表型,但直至如今相关的比较研究仍寥寥无几。在本研究中,运用免疫遗传学方法、热力学、凝集动力学和凝集图谱对不同样本进行了分析。共检测了近百例弱B样本,分属于29个家族,包括顺式AB,但不包括获得性B和Bh。运用这些方法可以证明,反应结构在不同家族间存在差异,但在同一家族内则严格相同。因此,每个样本都应以家族名称来命名;从实际应用角度来看,有必要达成一个简单的分类共识。于是寻找了最简单的标准,如玻片上的凝集速度、凝集图像、血清中抗B的有无等。随后区分出了三组:1)B3型,其凝集速度快(小于或等于30秒),呈现典型的混合视野凝集模式;血清中无抗B;2)Bx型,其凝集速度慢(大于或等于30秒),通常呈现弱凝集模式;血清中有弱抗B;3)Bel型,完全不发生凝集;通过固定洗脱试验证明膜上存在B抗原;在这三种情况下,遗传学研究证明该表型是由ABO基因座上的一个等位基因所致,Bel组是最具“争议性”的;当唾液中能证明有大量B物质分泌,且该表型被证明是以显性性状遗传时,可提出命名为Bm。相反,当遗传学表明存在一个修饰基因时,根据修饰类型是显性还是隐性,可分别使用Bel mod或By命名。仅一个样本似乎无法分类,其固定洗脱试验呈阳性,但只有5%至10%的红细胞发生凝集,呈现双群体模式;半乳糖基转移酶活性似乎正常,以及ΔH(20,000卡/摩尔)也正常。对于该特定样本,“弱B”这一命名似乎最为合适。除了这个案例外,所提出的实际分类似乎与所研究的表型非常契合。鉴于文献中报道的关于弱B表型的各种研究并未在同一实验室使用相同技术进行比较,因此将所提出的分类应用于不同报道的样本似乎并不现实。本研究纳入了一些已发表的样本,但它们的命名与之前的不同;令人惊讶的是,文献中仅描述了少数B3(新命名)型,这让人认为使用经典分组试验可能无法检测到它们,从而将其视为正常B型。当然,定义A³、Ax、Ael表型的标准与这里为B3、Bx、Bel提出的标准之间并不存在绝对的对应关系……

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