Rituximab in autoimmune thrombotic thrombocytopenic purpura: A success story

Eur J Intern Med. 2015 Nov;26(9):659-65. doi: 10.1016/j.ejim.2015.07.021. Epub 2015 Aug 17.


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?

Keywords: ADAMTS13; Immunotherapy; Rituximab; Thrombotic thrombocytopenic purpura.

Publication types

  • Review

MeSH terms

  • ADAM Proteins / deficiency
  • ADAM Proteins / metabolism*
  • ADAMTS13 Protein
  • Antirheumatic Agents / administration & dosage*
  • Antirheumatic Agents / adverse effects
  • B-Lymphocytes / drug effects
  • Disease Progression
  • Humans
  • Immunotherapy / methods
  • Plasma Exchange
  • Purpura, Thrombotic Thrombocytopenic / therapy*
  • Recurrence
  • Rituximab / administration & dosage*
  • Rituximab / adverse effects


  • Antirheumatic Agents
  • Rituximab
  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human