Expression of CTLA-4 and CD28 in hemophilia A children with FⅧ inhibitor

doi:10.3969/j.issn.1006-7795.2018.03.001

Abstract

Abstract: Objective To explore the expression of cytotoxic T lymphocyte-associated antigen-4 (CTLA-4) and CD28 in children with hemophilia A inhibitor.Methods Data of a total of 58 children with hemophilia A (FⅧ:C ≤ 2%) in the Beijing Children's Hospital, Capital Medical University from December 1, 2016 to January 1, 2018 were collected, 28 cases were positive for the inhibitor and 30 cases were negative.Improved Nijmegen assay was used to test inhibitor titer and flow cytometry to test percentage of CD3、CD4、CTLA-4 and CD28 on T cells in peripheral blood. We analyzed the expression of CTLA-4/CD28 axis and CTLA-4+ Th cells, CD28+ Th cells of children with hemophilia A inhibitor positive and negative, and to determine the difference.Results The proportion of CTLA-4+Th cells in peripheral blood of children with FⅧ inhibitor was 2.95%(1.41%,5.50%), and negative group was 4.95%(2.20%,7.70%). The positive group was significantly lower than that of negative group (P=0.038). The expression of CD28+Th cells was not different. The CTLA-4/CD28 cell in peripheral blood of the positive group was 0.03 (0.01,0.06), and the negative group was 0.05 (0.02,0.08). the positive group was significantly lower than that of the negative group (P=0.042). There was no significant difference between the two groups in low titer group and high titer group.Conclusion The results of this study showed that expression of CTLA-4+T cells in peripheral blood of children with hemophilia A inhibitor positive was significantly lower than that in the negative children. We infer that the imbalance of CTLA-4/CD28 axis results in the development of inhibitor, while the insufficient expression of CTLA-4 is the main factor. Insufficient expression of CTLA-4 may be one of the important immunosuppressive effects of inhibitors generation.

Key words: CTLA-4/CD28, hemophilia A, inhibitor

CLC Number:

R725.5

Li Yang, Chen Zhenping, Li Gang, Zhen Yingzi, Wu Xinyi, Wu Runhui. Expression of CTLA-4 and CD28 in hemophilia A children with FⅧ inhibitor[J]. Journal of Capital Medical University, 2018, 39(3): 309-313.

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URL: https://journal03.magtech.org.cn/Jweb_sdykdxxb/EN/10.3969/j.issn.1006-7795.2018.03.001

https://journal03.magtech.org.cn/Jweb_sdykdxxb/EN/Y2018/V39/I3/309

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