Concentration and Subclass Distribution of Anti-ADAMTS13 IgG Autoantibodies in Different Stages of Acquired Idiopathic Thrombotic Thrombocytopenic Purpura.

摘要: Background: The acquired form of idiopathic thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease, in which the underlying deficiency ADAMTS13 protease caused by autoantibodies, predominantly IgG isotype. Certain HLA-DR-DQ haplotypes were associated with risk developing TTP. Objectives: To investigate development ADAMTS13-specific antibody response during course we analyzed concentration, subclass distribution, and inhibitory potential anti-ADAMTS13 autoantibodies samples TTP patients drawn first acute phase, remission, relapse. Additionally, compared levels between carrying not protective haplotypes. Patients methods: We determined concentration distribution 101 antibody-positive 81 ELISA methods. presence semi-quantitative amount inhibitors 97 100 deficient samples, specific 49 selected mixing activity assays. typing haplotype prediction performed 70 above patients. Results: found that IgG1 IgG4 predominant subclasses, present almost all samples. While was dominant half taken episode, or following a correlated subclass. Anti-ADAMTS13 antibodies IgG4-dominant had higher potentials than IgG1-dominant independently disease stage. Interestingly, DR7-DQ2 DR13-DQ6 levels. Conclusion: Our results indicate becomes subtype at some point course, apparently before relapse, parallel to increase autoantibodies. Furthermore, association genetic background