Ferrari, Silvia; Palavra, Kristina; Gruber, Bernadette; Kremer Hovinga, Johanna Anna; Knöbl, Paul; Caron, Claudine; Cromwell, Caroline; Aledort, Louis; Plaimauer, Barbara; Turecek, Peter L.; Rottensteiner, Hanspeter; Scheiflinger, Friedrich (2014). Persistence of circulating ADAMTS13-specific immune complexes in patients with acquired thrombotic thrombocytopenic purpura. Haematologica - the hematology journal, 99(4), pp. 779-787. Ferrata-Storti Foundation 10.3324/haematol.2013.094151
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Anti-ADAMTS13 autoantibodies are the main cause of acquired thrombotic thrombocytopenic purpura. Binding of these antibodies to ADAMTS13 eventually results in the formation of antigen-antibody immune complexes. Circulating ADAMTS13-specific immune complexes have been described in acquired thrombotic thrombocytopenic purpura patients, however, the prevalence and persistence of these immune complexes over time has hitherto remained elusive. Here, we analyzed a large cohort of patients with acquired thrombotic thrombocytopenic purpura for the presence of free and complexed anti-ADAMTS13 antibodies. In the acute phase (n=68), 100% of patients had free IgG antibodies and 97% had ADAMTS13-specific immune complexes. In remission (n=28), 75% of patients had free antibodies (mainly IgG) and 93% had ADAMTS13-specific immune complexes. Free antibodies were mainly of subclasses IgG1 and IgG4, whereas IgG4 was by far the most prevalent in ADAMTS13-specific immune complexes. Comparison of ADAMTS13 inhibitor and anti-ADAMTS13 IgG (total and subclasses) antibody titers in acute phase and in remission samples showed a statistically significant decrease in all parameters in remission. Although non-significant, a trend towards reduced or undetectable titers in remission was also observed for ADAMTS13-specific immune complexes of subclasses IgG1, IgG2 and IgG3. For IgG4, no such trend was discernible; IgG4 immune complexes persisted over years, even in patients who had been treated with rituximab and who showed no features suggesting relapse.
Item Type: |
Journal Article (Original Article) |
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Division/Institute: |
04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory |
UniBE Contributor: |
Kremer Hovinga Strebel, Johanna Anna |
Subjects: |
600 Technology > 610 Medicine & health |
ISSN: |
0390-6078 |
Publisher: |
Ferrata-Storti Foundation |
Language: |
English |
Submitter: |
Verena Zwahlen |
Date Deposited: |
16 Jun 2014 10:08 |
Last Modified: |
02 Mar 2023 23:24 |
Publisher DOI: |
10.3324/haematol.2013.094151 |
PubMed ID: |
24241492 |
Uncontrolled Keywords: |
ADAMTS13, Thrombotic Thrombocytopenic Purpura acquired TTP anti-ADAMTS13 antibodies immune complexes |
BORIS DOI: |
10.7892/boris.48711 |
URI: |
https://boris.unibe.ch/id/eprint/48711 |
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