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The role of human leukocyte antigens as predisposing and/or protective factors in patients with idiopathic thrombotic thrombocytopenic purpura

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Abstract

Fifty-four adult German patients suffering from idiopathic thrombotic thrombocytopenic purpura (TTP) have been examined for HLA class II. All patients presented autoantibodies against ADAMTS13 and ADAMTS13 activity levels <5%. Blood samples have been analyzed for HLA-DRB1 and DQB1 alleles using sequence-specific primer PCR and sequence-specific oligonucleotide PCR. Reference data of German bone marrow and blood donors were obtained from www.allelefrequencies.net. The results were evaluated employing two-sided binomial tests, and p values were corrected using the Benjamini–Hochberg procedure. A significant accumulation for DQB1*02:02 (p < 0.001) and DRB1*11 (p = 0.003) was found within the patient group. Twenty percent (DQB1*02:02) or 48.1% (DRB1*11) of TTP patients were tested positive for the particular HLA antigen compared to 1.2% (DQB1*02:02) or 23.5% (DRB1*11) in the control group. A tendency for a reduced occurrence of HLA-DRB1*04 was revealed (7.4% in patients compared to 24.6% in controls). An association between the HLA antigens DQB1*02:02 and DRB1*11 and disease susceptibility for idiopathic TTP has been found. A higher risk for disease outbreak within persons carrying the mentioned alleles can be assumed. The reduced occurrence of HLA-DRB1*04 in TTP patients indicates a possible protective effect of this HLA allele in disease development.

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Authors and Affiliations

  1. Department of Hematology, University Medical Center, Mainz, Germany

    Marie-Luise John & Inge Scharrer

  2. Center for Transfusion Medicine, University Medical Center, Mainz, Germany

    Walter Hitzler

  3. III. Medizinische Klinik, Abteilung für Hämatologie, Langenbeckstraße 1, 55131, Mainz, Germany

    Inge Scharrer

Authors
  1. Marie-Luise John
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  2. Walter Hitzler
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  3. Inge Scharrer
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Correspondence to Inge Scharrer.

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John, ML., Hitzler, W. & Scharrer, I. The role of human leukocyte antigens as predisposing and/or protective factors in patients with idiopathic thrombotic thrombocytopenic purpura. Ann Hematol 91, 507–510 (2012). https://doi.org/10.1007/s00277-011-1384-z

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  • DOI: https://doi.org/10.1007/s00277-011-1384-z

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