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Blood, 15 September 2005, Vol. 106, No. 6, pp. 1932-1937.
Prepublished online as a Blood First Edition Paper on June 2, 2005; DOI 10.1182/blood-2005-03-0848.
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CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS
Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases
Fadi Fakhouri,
Jean-Paul Vernant,
Agnès Veyradier,
Martine Wolf,
Gilles Kaplanski,
Raynald Binaut,
Manfred Rieger,
Friedrich Scheiflinger,
Pascale Poullin,
Benjamin Deroure,
Richard Delarue,
Philippe Lesavre,
Philippe Vanhille,
Olivier Hermine,
Giuseppe Remuzzi, and
Jean-Pierre Grünfeld
From the Service de Néphrologie, Hôpital Necker, Université Paris V-René Descartes, Paris, France; Service d'Hématologie, Hôpital de la Pitié-Salpêtrière, Paris, France; Service d'Hématologie biologique, Hôpital Antoine Béclère, Clamart, France; Institut National de la Santé et de la Recherche Médicale (INSERM) U143, Le Kremlin-Bicêtre, France; Service de Médecine interne, Hôpital de La Conception, Marseille, France; Service de Médecine interne et de Néphrologie, Hôpital de Valenciennes, Valenciennes, France; Baxter Biomedical Research Center, Orth/Donau, Austria; Service d'Hémaphérèse et Autotransfusion, Hôpital de La Conception, Marseille, France; Service d'Hématologie, Hôpital Necker-Enfants Malades, Paris, France; INSERM U507, Paris, France; and Mario Negri Institute for Pharmacological Research, Bergamo, Italy.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that occurs mainly in young adults. Acquired cases are usually a result of antibodies directed against ADAMTS13 (a disintegrin-like and metalloprotease [reprolysin type] with thrombospondin type 1 motif 13), a protease that cleaves the von Willebrand factor multimers. Prognosis has been improved by plasma therapy, but some acute severe forms are refractory to this treatment and achieving a sustained remission is still a challenge in chronic relapsing forms. We therefore conducted a multicentric open-label prospective trial to test the efficacy of rituximab, an anti–B-cell monoclonal antibody, as a curative and prophylactic treatment in patients with TTP as a result of anti-ADAMTS13 antibodies. Six patients were included during an acute refractory TTP episode. Five patients with severe relapsing TTP and persistent anti-ADAMTS13 antibodies were prophylactically treated during remission. All patients received 4 weekly infusions of rituximab. The target of treatment was to restore a significant ADAMTS13 plasma activity (> 10%). Treatment with rituximab led to clinical remission in all cases of acute refractory TTP. In all patients, anti-ADAMTS13 antibodies disappeared, and a significant (18%-75%) plasma ADAMTS13 activity was detected following treatment. Tolerance of rituximab was good. Rituximab is a promising first-line immunosuppressive treatment in patients with acute refractory and severe relapsing TTP related to anti-ADAMTS13 antibodies.
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