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[儿童特发性血小板减少性紫癜与HLA - DRB1等位基因的关系]

[Relationship between HLA-DRB1 alleles and idopathic thrombocytopenic purpura in children].

作者信息

Wang Hongmei, Shen Baijun, Yan Wenying, Zhu Na, Qi Hongying, Hou Huaishui

机构信息

Department of Paediatrics, Shandong Provincial Hospital, Jinan 250021, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2002 Sep;23(9):466-9.

Abstract

OBJECTIVE

To study the relationship between HLA-DRB1 alleles and idiopathic thrombocytopenic purpura (ITP) in children.

METHODS

PCR-SSO was used to identify DRB1 alleles of 42 children with ITP. Among them, anti-GPIIb/IIIa and anti-GPIb/IX autoantibody were detected in 36 cases by modified monoclonal antibody specific immobilization of platelet antigens (MAIPA).

RESULTS

(1) Compared with healthy controls, HLA-DRB1 * 17 was significantly increased (relative risk = 2.76, P < 0.05, etiologic factor = 0.106 4) and HLA-DRB1 * 1202 decreased (relative risk = 0.20, P < 0.025, prophylactic factor = 0.761 6) in children with ITP. (2) In comparison with patients with good response to steroids and IgG therapy, HLA-DRB1 * 11 was significantly increased (P < 0.025) in patients with a poor response, furthermore, most (5/6) of HLA-DRB1 * 11-positive patients were female teen-ager. (3) Twenty-seven patients (75%) had anti-GPIIb/IIIa and seventeen (47.22%) had anti-GPIb/IX autoantibodies, the positivity rates of both anti-GPIIb/IIIa (P = 0.02) and anti-GPIb/IX (P = 0.01) were associated with HLA-DRB1 * 02. However, the pos./itivity rates of autoantibodies between refractory and non-refractory patients showed no significant difference.

CONCLUSION

(1) The DRB1 * 17 seems to predict susceptibility to ITP in children, while DRB1 * 1202 appears to be protective to against ITP. (2) The DRB1 * 11 plays an important role in resistance to steroid and IgG therapy in children with ITP. (3) It seems that the response to the antigenic epitope of GPIIb/IIIa and GPIb/IX is restricted by DRB1 * 02, while the presence of the autoantibodies couldn't predict prognosis. Our preliminary findings indicate that genetic factors influence the clinical course of ITP, but its exact mechanism needs to be further investigated.

摘要

目的

研究HLA - DRB1等位基因与儿童特发性血小板减少性紫癜(ITP)之间的关系。

方法

采用聚合酶链反应-序列特异性寡核苷酸探针技术(PCR - SSO)鉴定42例ITP患儿的DRB1等位基因。其中36例采用改良的血小板抗原单克隆抗体特异性固定法(MAIPA)检测抗GPIIb/IIIa和抗GPIb/IX自身抗体。

结果

(1)与健康对照相比,ITP患儿中HLA - DRB1 * 17显著增加(相对危险度=2.76,P < 0.05,病因系数=0.106 4),而HLA - DRB1 * 1202降低(相对危险度=0.20,P < 0.025,保护系数=0.761 6)。(2)与对类固醇和免疫球蛋白治疗反应良好的患者相比,反应较差的患者中HLA - DRB1 * 11显著增加(P < 0.025),此外,HLA - DRB1 * 11阳性的患者中大多数(5/6)为青少年女性。(3)27例患者(75%)有抗GPIIb/IIIa,17例(47.22%)有抗GPIb/IX自身抗体,抗GPIIb/IIIa(P = 0.02)和抗GPIb/IX(P = 0.01)的阳性率均与HLA - DRB1 * 02相关。然而,难治性和非难治性患者自身抗体的阳性率无显著差异。

结论

(1)DRB1 * 17似乎可预测儿童ITP的易感性,而DRB1 * 1202似乎对ITP有保护作用。(2)DRB1 * 11在ITP患儿对类固醇和免疫球蛋白治疗的抵抗中起重要作用。(3)似乎对GPIIb/IIIa和GPIb/IX抗原表位的反应受DRB1 * 02限制,而自身抗体的存在不能预测预后。我们的初步研究结果表明遗传因素影响ITP的临床病程,但其确切机制有待进一步研究。

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