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[慢性特发性血小板减少性紫癜自身抗体的克隆特征研究]

[Study of the clonal characteristics of autoantibodies in chronic idiopathic thrombocytopenic purpura].

作者信息

Hou Ming, Shi Yan, Peng Jun, Li Wei, Ma Dao-xin, Zhang Mao-hong

机构信息

Shandong University Qilu Hospital, Shandong 250012, China.

出版信息

Zhonghua Nei Ke Za Zhi. 2004 Feb;43(2):87-9.

Abstract

OBJECTIVE

To define the clonality characteristics of autoantibodies in chronic idiopathic thrombocytopenic purpura (ITP).

METHODS

The heavy-chain and light-chain phenotype composition of the glycoprotein (GP)-specific IgG antibodies were analysed with a modified monoclonal antibody specific immobilization of platelet antigens (MAIPA) technique. The immunoglobulin heavy-chain gene rearrangement was analysed by PCR amplification.

RESULTS

16 out of the 43 patient sera reacted with least one of the following five GPs, namely GPIIb/IIIa, GPIb, GPIa, GPIV and GPV. Eight of 11 (73%) GP-specific antibodies displayed a restricted heavy-chain phenotypes. 80% (16/20) of the GP-specific antibodies showed a restricted kappa or lambda light-chain phenotype. Moreover, in 6 patients the GP-specific antibodies were found to be both light-chain and heavy-chain restricte. Using PCR amplification of immunoglobulin heavy-chain genes, 3 patients displayed heavy-chain genes rearrangement.

CONCLUSION

The GP-specific autoantibodies are derived from a restricted number of B-cell clones in proportion of ITP patients.

摘要

目的

明确慢性特发性血小板减少性紫癜(ITP)中自身抗体的克隆性特征。

方法

采用改良的单克隆抗体特异性血小板抗原固定技术(MAIPA)分析糖蛋白(GP)特异性IgG抗体的重链和轻链表型组成。通过PCR扩增分析免疫球蛋白重链基因重排。

结果

43例患者血清中有16例与以下5种GP中的至少一种发生反应,即GPIIb/IIIa、GPIb、GPIa、GPIV和GPV。11例(73%)GP特异性抗体中有8例表现出受限的重链表型。80%(16/20)的GP特异性抗体表现出受限的κ或λ轻链表型。此外,在6例患者中发现GP特异性抗体的轻链和重链均受限。通过PCR扩增免疫球蛋白重链基因,3例患者出现重链基因重排。

结论

在一定比例的ITP患者中,GP特异性自身抗体来源于有限数量的B细胞克隆。

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