Published online 26 January 2008
Haematologica, Vol 93, Issue 2, 232-239 doi:10.3324/haematol.11739
Copyright © 2008 by Ferrata Storti Foundation
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Thrombosis

ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission

Flora Peyvandi1,, Silvia Lavoretano1, Roberta Palla1, Hendrik B. Feys2, Karen Vanhoorelbeke2, Tullia Battaglioli1, Carla Valsecchi1, Maria Teresa Canciani1, Fabrizio Fabris3, Samo Zver4, Marienn Réti5, Danijela Mikovic6, Mehran Karimi7, Gaetano Giuffrida8, Luca Laurenti9, Pier Mannuccio Mannucci1

1 Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, University of Milan, Department of Medicine and Medical Specialities, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Luigi Villa Foundation, Milan, Italy;
2 Laboratory for Thrombosis Research, IRC, KULeuven Campus Kortrijk, Kortrijk, Belgium;
3 Internal Medicine, Unit Department of Medical and Surgical Sciences, University of Padua, Padua, Italy;
4 Department of Haematology, University Medical Center, Ljubljana, Slovenija;
5 Department of Hematology and Stem Cell Transplantation, National Medical Center, Budapest, Hungary;
6 emostasis Department and Hemophilia Center, National Blood Transfusion Institute, Belgrade, Serbia;
7 Haemostasis and Thrombosis Unit, Haematology Research Centre, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
8 Division of Hematology and Bone Marrow Transplantation, Vittorio Emanuele Ferrarotto-S.Bambino Hospital, University of Catania, Catania, Italy;
9 Hematology Institute, Catholic University, Rome, Italy

Correspondence: Flora Peyvandi, MD, PhD, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, University of Milan, Via Pace, 9, 20122, Milan, Italy. E-mail: flora.peyvandi{at}unimi.it

Background: From 20 to 50% of patients who survive an acute episode of the acquired form of thrombotic thrombocytopenic purpura relapse but clinical and laboratory markers of recurrence are not well established.

Design and Methods: In 109 patients enrolled in an international registry we evaluated, in the frame of a retrospective cohort study, the predictive role of the metalloprotease ADAMTS13 as measured in plasma during remission. Anti-ADAMTS13 antibodies and von Willebrand factor were also evaluated in a smaller number of the same patients.

Results: Median values of ADAMTS13 activity and antigen were significantly lower in patients with recurrent thrombotic thrombocytopenic purpura than in those with no recurrence (activity: 12% vs. 41%; p=0.007; antigen: 36% vs. 58%; p=0.003). A severe deficiency of ADAMTS13 activity (10% or less) was associated with a higher likelihood of recurrence (odds ratio 2.9; 95% confidence interval 1.3 to 6.8; p=0.01). Anti-ADAMTS13 antibodies were also more prevalent in patients with recurrent thrombotic thrombocytopenic purpura (odds ratio 3.1; 95% confidence interval 1.4 to 7.3; p=0.006). The presence during remission of both severe ADAMTS13 deficiency and anti-ADAMTS13 antibodies increased the likelihood of recurrence 3.6 times (95% confidence interval 1.4 to 9.0; p=0.006). The presence of ultralarge von Willebrand factor multimers and of associated diseases or conditions did not increase recurrence.

Conclusions: Survivors of an acute episode of acquired thrombotic thrombocytopenic purpura with severely reduced levels of ADAMTS13 and/or with anti-ADAMTS13 antibodies during remission have an approximately three-fold greater likelihood of developing another episode of thrombotic thrombocytopenic purpura than patients with higher protease activity and no antibody.

Key words: thrombotic thrombocytopenic purpura, ADAMTS13, von Willebrand factor, risk factors, recurrence.


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Bernhard Lämmle, Johanna A. Kremer Hovinga, James N. George
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