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Blood, 1 August 2005, Vol. 106, No. 3, pp. 925-928.
Prepublished online as a Blood First Edition Paper on April 12, 2005; DOI 10.1182/blood-2004-12-4885.


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Submitted December 27, 2004
Accepted March 24, 2005

Rituximab prevents recurrence of thrombotic thrombocytopenic purpura: a case report

Miriam Galbusera, Elena Bresin, Marina Noris, Sara Gastoldi, Daniela Belotti, Cristina Capoferri, Erica Daina, Paolo Perseghin, Friedrich Scheiflinger, Fadi Fakhouri, Jean-Pierre Grunfeld, Enrico Pogliani, and Giuseppe Remuzzi*

Mario Negri Institute for Pharmacological Research, Clinical Research Center for Rare Diseases, Aldo e Cele Dacco, Ranica, Bergamo, Italy
Unit of Hematology, Ospedale San Gerardo, Monza, Italy
Immunohematology and Blood Transfusion Center, Ospedale San Gerardo, Monza, Italy
Baxter BioScience, Biomedical Research Center, Orth/Donau, Austria
Unit of Nephrology, Hospital Necker, Paris, France
Mario Negri Institute for Pharmacological Research, Clinical Research Center for Rare Diseases, Aldo e Cele Dacco, Ranica, Bergamo, Italy; Unit of Nephrology and Dialysis, Azienda Ospedaliera, Ospedali Riuniti di Bergamo, Bergamo, Italy

* Corresponding author; email: gremuzzi{at}marionegri.it.

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of small vessels that is associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS-13. The presence of anti-ADAMTS-13 autoantibodies is considered a factor predisposing to relapses. Despite close monitoring and intensive plasma treatment, in these patients acute episodes are still associated with substantial morbidity and mortality rates and the optimal therapeutic option should rather be prevention of relapses. This study was conducted in a patient with recurrent TTP due to high titers of ADAMTS-13 inhibitors, who used to have 2 relapses of TTP a year and compared the standard treatment plasma exchange with rituximab. Results documented that plasma exchange had only a small transient effect on ADAMTS-13 activity and inhibitors, on the contrary prophylaxis with rituximab was associated with disappearance of anti-ADAMTS-13 antibodies, a progressive recovery of protease activity, and allowed the patient to maintain a disease-free state for more than 2 year follow-up.


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J. E. Sadler
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura
Blood, July 1, 2008; 112(1): 11 - 18.
[Abstract] [Full Text] [PDF]



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