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再生障碍性贫血和范科尼贫血患者中HLA - A2及HLA - A2纯合子过量

[Excess of HLA-A2 and HLA-A2 homozygotes in patients with aplastic and Fanconi's anemias].

作者信息

Dausset J, Gluckman E, Lemarchand F, Nunez-Roldan A, Contu L, Hors J

出版信息

Nouv Rev Fr Hematol Blood Cells. 1977;18(2):315-24.

PMID:556339
Abstract

In 68 idiopathic aplastic anemia patients (65 HLA genotypes), HLA-A2 is slightly increased (p corrected less than 0.03) leading to a relative risk of 2.30. A slight excess of homozygotes for HLA-A was observed (22.2 vs. 14.09%), involving mostly the A2 antigen. In the siblings the patients were more frequently homozygote A2 than by chance (p less than 0.005). The same trends are observed in Fanconi's anemia = out of 18 patients, 7 are HLA-A homozygote (of whom 4 are A2). The hypothesis of the existence of one or several recessive genes involved in hematopoiesis situated close to HLA-A is advanced.

摘要

在68例特发性再生障碍性贫血患者(65种HLA基因型)中,HLA - A2略有增加(校正后p值小于0.03),相对风险为2.30。观察到HLA - A纯合子略有过量(22.2%对14.09%),主要涉及A2抗原。在同胞中,患者为A2纯合子的频率高于预期偶然发生率(p值小于0.005)。在范科尼贫血中也观察到相同趋势 = 在18例患者中,7例为HLA - A纯合子(其中4例为A2)。提出了存在一个或几个与造血相关的隐性基因且位于靠近HLA - A位置的假说。

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