What is the role of rituximab in the treatment of thrombotic thrombocytopenic purpura (TTP)?

Updated: Feb 18, 2019
  • Author: Theodore Wun, MD, FACP; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

Increasing evidence supports the use of the anti-CD20 monoclonal antibody rituximab in cases of TTP refractory to plasma exchange, with resolution of acute disease and prolonged remission. [10, 21, 22, 23] British guidelines recommend offering rituximab to patients with refractory or relapsing immune-mediated TTP, and considering rituximab as part of first-line therapy, along with plasma exchange and steroids, in acute idiopathic TTP with neurological/cardiac pathology, as those cases are associated with high mortality. [15]

Jestin et al reported that in patients with immune-mediated TTP in remission who have severe ADAMTS13 deficiency (activity < 10%), preemptive treatment with rituximab can often spur recovery of ADAMTS13 and reduce the frequency of relapses. Patients in whom severe ADAMTS13 deficiency failed to respond to an initial course of rituximab, or recurred after initial improvement, typically responded to retreatment with rituximab. [24]  


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