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Blood, 1 April 2007, Vol. 109, No. 7, pp. 2815-2822.
Prepublished online as a Blood First Edition Paper on December 12, 2006; DOI 10.1182/blood-2006-02-006064.
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Submitted February 27, 2006
Accepted November 20, 2006
Prognostic value of anti-ADAMTS13 antibodies features (Ig isotype, titer and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with an undetectable ADAMTS13 activity
Silvia Ferrari, Friedrich Scheiflinger, Manfred Rieger, Geert Mudde, Martine Wolf, Paul Coppo, Jean-Pierre Girma, Elie Azoulay, Christian Brun-Buisson, Fadi Fakhouri, Jean-Paul Mira, Eric Oksenhendler, Pascale Poullin, Eric Rondeau, Nicolas Schleinitz, Benoit Schlemmer, Jean-Louis Teboul, Philippe Vanhille, Jean-Paul Vernant, Dominique Meyer, and Agnes Veyradier*
Department of Discovery Research and Technical Assessment, Baxter Biosciences, Vienna, Austria
Service d'Hematologie biologique, Hopital Antoine Beclere, Clamart and Faculte de Medecine Paris, Le Kremlin-Bicetre, France
Service d'Hematologie Adultes, Hopital Saint-Antoine, Paris, France
Inserm Unite U770, Le Kremlin-Bicetre, France
Service de Reanimation Medicale, Hopital Saint-Louis, Paris, France
Service de Reanimation Medicale, Hopital Henri-Mondor, Creteil, France
Service de Nephrologie Adultes, Hopital Necker, Paris, France
Service de Reanimation Medicale, Hopital Cochin, Paris, France
Service d'Immunologie clinique, Hopital Saint-Louis, Paris, France
Centre d'Hemapherese, Hopital de la Conception, Marseille, France
Service de Nephrologie Adultes, Hopital Tenon, Paris, France
Service de Medecine Interne, Hopital de la Conception, Marseille, France
Service de Reanimation Medicale, Hopital de Bicetre, Le Kremlin-Bicetre, France
Service de Medecine interne, Nephrologie, Hospitalier de Valenciennes, Valenciennes, France
Service d'Hematologie Adultes, Hopital de la Pitie-Salpetriere, Paris, France
* Corresponding author; email: agnes.veyradier{at}abc.aphp.fr.
To study both the pathophysiologic and the prognostic value of ADAMTS13 in thrombotic microangiopathies (TMA), we enrolled a cohort of 35 adult patients combining a first acute episode of TMA, an undetectable (<5%) ADAMTS13 activity in plasma and no clinical background such as sepsis, cancer, HIV and transplantation. All patients were treated by steroids and plasma exchange and an 18-month follow-up was scheduled. Remission was obtained in 32 patients (91.4%) while 3 patients died (8.6%) after the first attack. At presentation, ADAMTS13 antigen was decreased in 32 patients (91.4%), an ADAMTS13 inhibitor was detectable in 31 patients (89%), an anti-ADAMTS13 IgG/M/A was present in 33 patients (94%). The 3 decedent patients were characterized by the association of several anti-ADAMTS13 Ig isotypes including very high IgA titers while mortality was independent of the ADAMTS13 inhibitor titer. In survivors, ADAMTS13 activity in remission increased to levels >15% in 19 patients (59%) but remained undetectable in 13 patients (41%). Six patients relapsed either once or twice (19%) during the follow-up. High levels of inhibitory anti-ADAMTS13 IgG at presentation were associated with the persistence of an undetectable ADAMTS13 activity in remission, the latter being predictive for relapses within an 18-month delay.
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