Rituximab in autoimmune thrombotic thrombocytopenic purpura: A success story

• Rituximab is recommended in acquired TTP with a suboptimal response with standard management.
• Rituximab should be considered in patients with severe ADAMTS13 deficiency during TTP remission.
• Rituximab in acquired TTP is associated with acceptable side effects.

Despite a significant improvement of thrombotic thrombocytopenic purpura (TTP) prognosis since the use of plasma exchange, morbidity and mortality remained significant because of poor response to standard treatment or exacerbations and relapses. Rituximab, a chimeric monoclonal antibody directed against the B-lymphocyte CD20 antigen, has shown a particular interest in this indication. Recent studies also reported strong evidence for its efficiency in the prevention of relapses. This review addresses these recent progresses and still opened questions in this topic: should rituximab be proposed in all patients at the acute phase? Should all patients benefit from a preemptive treatment? Is the infectious risk acceptable in this context?