[血小板无力症、PLA1抗原与抗血小板无力症抗体]

[Glanzmann thrombasthenia, PLA1 antigen and anti-Glanzmann antibody].

作者信息

Muller J Y, Patereau C, Soulier J P

出版信息

Rev Fr Transfus Immunohematol. 1978 Dec;21(5):1069-78. doi: 10.1016/s0338-4535(78)80004-x.

Abstract

Six cases of Glanzmann's thrombasthenia were studied using a platelet indirect radioactive Coombs (PIRC). In serum of two among six patients, an antibody was found, which reacted positively with all platelets except those of thrombasthenic patients. Such "anti-public" antibody which shortens the life of transfused platelets is a very serious complication of Glanzmann's thrombasthenia. Attempts to define the PLA group of the six Glanzmann patients, with human allo antisera recognizing PLA1 antigen, gave negative results. Three hypothesis were discussed: (1) Interference in the test of the aggregation defect of thrombasthenic platelets. However, anti-HLA antibodies were normally fixed in the PIRC test. (2) PLA2 gene and Glanzmann gene have a strong gametic association and all Glanzmann's patients are PLA1 negative. (3) Glanzmann gene is coding for the PLA gene substrate. In such case, thrombasthenic patients might be genetically PLA1 positive and phenotypically PLA1 negative. Moreover they would also be PLA2 negative, this could not be tested due to the lack of anti-PLA2 antiserum.

摘要

运用血小板间接放射性库姆斯试验（PIRC）对6例Glanzmann血小板无力症患者进行了研究。在6例患者中的2例血清中，发现了一种抗体，该抗体与除血小板无力症患者血小板外的所有血小板均呈阳性反应。这种缩短输注血小板寿命的“抗公共”抗体是Glanzmann血小板无力症的一种非常严重的并发症。尝试用人源同种异体抗血清识别PLA1抗原，以确定6例Glanzmann患者的PLA血型组，但结果为阴性。讨论了三种假设：（1）干扰血小板无力症血小板聚集缺陷的检测。然而，抗HLA抗体在PIRC试验中通常是固定的。（2）PLA2基因与Glanzmann基因有很强的配子关联，所有Glanzmann患者均为PLA1阴性。（3）Glanzmann基因编码PLA基因底物。在这种情况下，血小板无力症患者在基因上可能是PLA1阳性，而在表型上是PLA1阴性。此外，他们也可能是PLA2阴性，由于缺乏抗PLA2抗血清，无法对此进行检测。