Highlights
- •Clinical value of ADAMTS13-specific circulating immune complexes (CICs) was assessed.
- •CICs level at the first TTP event did not reflect the severity of the acute episode.
- •CICs seemed to confer an increased relapse risk within 2 years from first TTP event.
- •CICs measurement may help to predict TTP recurrence in high-risk patients.
Abstract
Background
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy
due to the development of autoantibodies against the VWF-cleaving protease ADAMTS13.
ADAMTS13-specific circulating immune complexes (CICs) have been described in patients
with acquired TTP, but their clinical relevance remained to be established. The aim
of this study was to assess the association between ADAMTS13-specific CICs and ADAMTS13-related
measurements, clinical and laboratory markers of disease severity, and occurrence
of TTP relapse, in autoimmune TTP patients.
Material and methods
We measured ADAMTS13-specific CICs in 51 patients with severe ADAMTS13 deficiency
and anti-ADAMTS13 autoantibodies, at the first episode of acquired TTP. The associations
between ADAMTS13-specific CICs and the variables of interest were assessed by linear,
logistic and Cox proportional hazard regression models, where appropriate.
Results
The prevalence of ADAMTS13-specific CICs in patients experiencing the first TTP episode
was 39% (95% confidence intervals [CI]: 26–52%). ADAMTS13-specific CICs were not associated
neither with laboratory markers of disease severity, nor with patterns of clinical
presentation. Conversely, among 45 survivors, a positive association was found between
the presence of ADAMTS13-specific CICs and the risk of recurrence within 2 years after the first TTP episode (adjusted hazard ratio, 3.4 [95% CI: 0.9 to 13.5]).
Conclusions
ADAMTS13-specific CICs seem to be able to predict the recurrence of acute TTP episodes
in the first 2 years after disease onset. Therefore, their measurement might be used as a tool to
stratify the risk of disease relapse, with potential influence on surveillance and
therapeutic choices during remission phase.
Keywords
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Article info
Publication history
Published online: November 22, 2016
Accepted:
November 2,
2016
Received in revised form:
October 31,
2016
Received:
September 2,
2016
Identification
Copyright
© 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
