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[Study on the relations between HLA-DRB1 alleles and idiopathic thrombocytopenic purpura in children]
Hongmei Wang1, Baijun Shen, Wenying Yan
1Department of Paediatrics, Shandong Provincial Hospital, Jinan, Shandong, 250021 P.R.China. whtwhm@263.net
Insights
Human leukocyte antigen-DRB1 (HLA-DRB1) allele HLA-DRB1*17 is associated with increased susceptibility to childhood idiopathic thrombocytopenic purpura (ITP), while HLA-DRB1*1202 may be protective. HLA-DRB1*11 influences treatment resistance in pediatric ITP patients.
Area of Science:
- Immunogenetics
- Pediatric Hematology
- Autoimmune Diseases
Context:
- Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by low platelet counts.
- The role of human leukocyte antigen (HLA) genes in the pathogenesis of ITP is increasingly recognized.
- Understanding genetic predispositions can aid in predicting disease course and treatment response.
Purpose:
- To investigate the association between specific HLA-DRB1 alleles and the susceptibility and clinical course of ITP in children.
- To explore the correlation between HLA-DRB1 alleles and autoantibody profiles in pediatric ITP.
- To determine if HLA-DRB1 alleles influence response to standard ITP therapies.
Summary:
- This study identified specific HLA-DRB1 alleles associated with ITP in children using PCR-SSO.
- HLA-DRB1*17 was linked to increased ITP susceptibility, while HLA-DRB1*1202 showed a protective effect.
- HLA-DRB1*11 was associated with poor response to steroids and IVIgG, particularly in female adolescents. HLA-DRB1*02 correlated with anti-GPIIb/IIIa and anti-GPIb/Ix autoantibody positivity.
Impact:
- Findings suggest that HLA-DRB1 alleles are significant genetic factors influencing ITP development and treatment outcomes in children.
- Identifies HLA-DRB1*17 as a potential susceptibility marker and HLA-DRB1*1202 as a protective factor for childhood ITP.
- Highlights the role of HLA-DRB1*11 in steroid/IVIgG resistance, guiding personalized treatment strategies for pediatric ITP.
Objective:
To gain an insight into the relations between human leukocyte antigen-DRB1 (HLA-DRB1) alleles and idiopathic thrombocytopenic purpura (ITP) in children.
Methods:
Polymerase chain reaction-sequence specific oligonucleotide (PCR-SSO) was used to identify DRB1 alleles of 42 children with ITP. Among them, 36 were identified for anti-GPIIb/IIIa and anti-GPIb/Ix autoantibody by modified monoclonal antibody specific immobilization of platelet antigens.
Results:
Compared with health controls, the frequency of HLA-DRB1*17 significantly increased (P<0.05, relative risk=2.76, etiologic factor=0.1064) and the frequency of HLA-DRB1*1202 significantly decreased (P<0.025, relative risk=0.20, prophylactic factor=0.7616) in children with ITP. In comparison with patients of good response to steroids and IVIgG therapy, the frequency of HLA DRB1*11 significantly increased (Chi-square=6.091, P<0.025) in patients with a poor response, furthermore, the most of HLA-DRB1*11 positive patients were female teen-agers. Twenty-seven patients (75%) had anti GPIIb/IIIa and seventeen (47.22%) had anti_GPIb/Ix autoantibodies. The positivities of both anti_GP IIb/IIIa (P=0.02) and anti-GPIb/Ix (P=0.01) were associated with HLA-D RB1*02. However, the positivity of autoantibodies between refractory and non-refractory patients showed no significant difference.
Conclusion:
The allele of HLA-DRB1*17 seems to predict susceptibility of ITP in children, while HLA-DRB1*1202 appears to be protective to ITP. The allele of HLA DRB1*11 plays an important role in resistance to steroid and IgG therapy in children with ITP. It seems that the response to the antigenic epitope of GPIIb/IIIa and GPIb/Ix is restricted by HLA-DRB1*02, while the presence of the antibodies could not predict prognosis. In conclusion, the above preliminary findings indicate that genetic factors influence the clinical course of ITP, but the exact mechanism needs to be investigated further.
