A NEW LOW-FREQUENCY PLATELET ALLOANTIGEN, VA(A), ON GLYCOPROTEIN-IIBIIIA ASSOCIATED WITH NEONATAL ALLOIMMUNE THROMBOCYTOPENIA
A NEW LOW-FREQUENCY PLATELET ALLOANTIGEN, VA(A), ON GLYCOPROTEIN-IIBIIIA ASSOCIATED WITH NEONATAL ALLOIMMUNE THROMBOCYTOPENIA
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DOI：
10.1111/j.1365-3148.1992.tb00131.x
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发表时间：
1992-03-01
影响因子：
1.5
通讯作者：
KERO, P
中科院分区：
文献类型：
--
作者：
KEKOMAKI, R;RAIVIO, P;KERO, P
我们描述了血小板同种异体免疫引起严重的血小板减少症的新生儿，只能通过测试父亲的血小板检测。使用抗糖蛋白（GP）IIbIIIa复合物（AP 2和2G 12）的单克隆抗体，通过单克隆抗体固定血小板蛋白测定（MAIPA）鉴定血小板特异性抗体。先前描述的GPIIbIIIa复合物上的同种异体抗原系统（HPA 1、HPA 3或HPA 4）不负责该反应。此外，新描述的血小板特异性抗原Sr（a）与该抗原不相同。因此，该抗原不同于所有已知的血小板同种异体抗原，并命名为Va（a）。抗原存在于患病儿童的血小板上。家族研究表明，血小板抗原作为常染色体显性遗传性状在三代中传递。在对250名献血者进行的人群研究中未发现Va（a +）个体，这表明该抗原在芬兰人群中的频率较低。免疫印迹分析未检测到Va抗原，这表明表位可能不是线性肽结构。抗原也被血小板溶解破坏。已知血小板的凝血酶活化可增加血小板上GPIIbIIIa的表达，但不会增加抗-Va（a）抗体结合位点的数量，其增加程度与抗-HPA 1a结合所观察到的程度相同。
We describe platelet alloimmunization which caused severe thrombocytopenia in a neonate and could only be detected by testing the father's platelets. The platelet-specific antibodies were identified by a monoclonal antibody-immobilized platelet protein assay (MAIPA) using monoclonal antibodies against glycoprotein (GP) IIbIIIa complex (AP2 and 2G12). The previously described alloantigen systems on the GPIIbIIIa complex (HPA 1, HPA 3 or HPA 4) were not responsible for the reaction. In addition the newly described private platelet antigen Sr(a) was not identical to the antigen. The antigen is therefore different from all known platelet alloantigens and was designated Va(a). The antigen was present on the platelets of the affected child. Family studies showed that the platelet antigen was transmitted as an autosomal dominant trait in three generations. No Va (a +) individuals were found in a population study of 250 blood donors, which indicates that the antigen is of low frequency in the Finnish population. The Va antigen was not detectable by immunoblot analysis, which suggests that the epitope may not be a linear peptide structure. The antigen was also destroyed by solubilization of platelets. Thrombin activation of platelets, known to increase the expression of GPIIbIIIa on platelets, did not increase the number of binding sites for anti-Va(a) antibodies to the extent observed with anti-HPA 1a binding.