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Abstract: ASN Annual Meeting -- Philadelphia |
Fakhouri F, Vernant J, Veyradier A, Wolf M, et al
Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13 deficient-thrombotic thrombocytopenic purpura: A study of 11 cases.
ASN Annual Meeting -- Philadelphia
J Am Soc Nephrol (Nov) 16:556 2005
Thrombotic thrombocytopenic purpura (TTP) is a life threatening disease occurring mainly in young adults. Acquired cases are usually due to antibodies directed against ADAMTS13, 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 in order to test the efficacy of rituximab, an anti-B cell monoclonal antibody, as a curative and prophylactic treatment in patients with TTP due to 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.
© Copyright 2005-2006, American Society of Nephrology. Reproduced with permission.
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