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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.
HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Rituximab prevents recurrence of thrombotic thrombocytopenic purpura: a case reportFrom the Mario Negri Institute for Pharmacological Research, Clinical Research Center for Rare Diseases, Aldo e Cele Daccò, Villa Camozzi-Ranica; the Unit of Hematology and the Immunohematology and Blood Trasfusion Center, Ospedale San Gerardo, Monza, Italy; Baxter BioScience, Biomedical Research Center, Uferstrasse 15, A-2304 Orth/Donau, Austria; the Unit of Nephrology, Hospital Necker, Paris, France; and the Unit of Nephrology and Dialysis, Azienda Ospedaliera, Ospedali Riuniti di Bergamo, Italy.
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of small vessels that is associated with deficiency of the von Willebrand factorcleaving protease, ADAMTS13. The presence of anti-ADAMTS13 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 be prevention of relapses. This study was conducted in a patient with recurrent TTP due to high titers of ADAMTS13 inhibitors, who used to have 2 relapses of TTP a year. The study compared the standard treatment plasma exchange with rituximab. Results documented that plasma exchange had only a small transient effect on ADAMTS13 activity and inhibitors; on the contrary, prophylaxis with rituximab was associated with disappearance of anti-ADAMTS13 antibodies, a progressive recovery of protease activity, and it allowed the patient to maintain a disease-free state during a more than 2-year follow-up.
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