van Leeuwen E F, von dem Borne A E, von Riesz L E, Nijenhuis L E, Engelfriet C P
Blood. 1981 Jan;57(1):49-54.
The platelets of 11 patients with Glanzmann's thrombasthenia and their nearest family members were studied for the expression of the platelet-specific alloantigens of the Zw-, Ko- and Bak systems. The strength of the expression of the Zwa antigen was diminished on the platelets of 3 patients, and the antigen was absent from the platelets of the other 8. The platelets of none of the patients reacted with anti-Zwb serum. Therefore, Glanzmann's thrombasthenia is probably a "Zw-null disease." The expression of the Zwa antigen on the platelets of all the relatives was normal, as indicated on the cytoflurograph. Investigations on the expression of the Koa antigen were complicated by agglutinations of the platelets from genetically Koa-negative thrombasthenic patients with the anti-Koa serum. The Kob antigen was normally expressed. The Baka antigen was absent from the platelets of all thrombasthenic patients and a relatively high percentage of the relatives. A close association between the Glanzmann gene and the Bak(a-) gene is assumed on statistical grounds. Thrombasthenic platelets showed no reaction with EDTA-dependent antibodies, which are reactive with all normal platelets. Owing to immunization by multiple blood and platelet transfusions, serum samples of most patients studied contained HLA antibodies and platelet-specific alloantibodies. However, antibodies directed against the Zw-antigen-bearing glycoproteins were detected in the serum of only one patient and, therefore, seem to be rare.
对11例Glanzmann血小板无力症患者及其近亲的血小板进行了研究，以检测Zw、Ko和Bak系统血小板特异性同种抗原的表达情况。3例患者血小板上Zwa抗原的表达强度减弱，另外8例患者血小板上则无该抗原。所有患者的血小板均不与抗Zwb血清发生反应。因此，Glanzmann血小板无力症可能是一种“Zw缺失病”。细胞荧光图显示，所有亲属血小板上Zwa抗原的表达均正常。对Koa抗原表达的研究因抗Koa血清使遗传性Koa阴性血小板无力症患者的血小板发生凝集而变得复杂。Kob抗原表达正常。所有血小板无力症患者及相当比例的亲属血小板上均无Baka抗原。基于统计学依据，推测Glanzmann基因与Bak(a-)基因之间存在密切关联。血小板无力症患者的血小板不与EDTA依赖性抗体发生反应，而这些抗体可与所有正常血小板发生反应。由于多次输血和输注血小板导致免疫，大多数研究患者的血清样本中含有HLA抗体和血小板特异性同种抗体。然而，仅在1例患者的血清中检测到针对携带Zw抗原的糖蛋白的抗体，因此这种抗体似乎很罕见。
