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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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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY
Prognostic value of anti-ADAMTS13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS13 activity
Silvia Ferrari1,
Friedrich Scheiflinger1,
Manfred Rieger1,
Geert Mudde1,
Martine Wolf2,3,
Paul Coppo4,
Jean-Pierre Girma3,
Elie Azoulay5,
Christian Brun-Buisson6,
Fadi Fakhouri7,
Jean-Paul Mira8,
Eric Oksenhendler9,
Pascale Poullin10,
Eric Rondeau11,
Nicolas Schleinitz12,
Benoit Schlemmer5,
Jean-Louis Teboul13,
Philippe Vanhille14,
Jean-Paul Vernant15,
Dominique Meyer2,3,
Agnès Veyradier, for the French and Clinical Biological Network on Adult Thrombotic Microangiopathies2,3
1 Baxter Bioscience, Department of Discovery Research and Technical Assessment, Vienna, Austria;
2 Service d'Hématologie Biologique, Hôpital Antoine-Béclère, Clamart and Faculté de Médecine Paris 11, Le Kremlin-Bicêtre, France;
3 Institut National de la Santé et de la Recherche Médicale (Inserm) Unité 770, Le Kremlin Bicêtre, France;
4 Service d'Hématologie Adultes, Hôpital Saint-Antoine, Paris, France;
5 Service de Réanimation Médicale, Hôpital Saint-Louis, Paris, France;
6 Service de Réanimation Médicale, Hôpital Henri-Mondor, Créteil, France;
7 Service de Néphrologie Adultes, Hôpital Necker, Paris, France;
8 Service de Réanimation Médicale, Hôpital Cochin, Paris, France;
9 Service d'Immunologie Clinique, Hôpital Saint-Louis, Paris, France;
10 Centre d'Hémaphérèse, Hôpital de la Conception, Marseille, France;
11 Service de Néphrologie Adultes, Hôpital Tenon, Paris, France;
12 Service de Médecine Interne, Hôpital de la Conception, Marseille, France;
13 Service de Réanimation Médicale, Hôpital de Bicêtre, Le Kremlin Bicêtre, France;
14 Service de Médecine Interne, Néphrologie, Hôpital de Valenciennes, France;
15 Service d'Hématologie Adultes, Hôpital de la Pitié-Salpêtrière, Paris, France
To study both the pathophysiologic and the prognostic value of ADAMTS13 in thrombotic microangiopathies (TMAs), we enrolled a cohort of 35 adult patients combining a first acute episode of TMA, an undetectable (below 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%), and 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%), and an anti-ADAMTS13 IgG/IgM/IgA 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 above 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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