The role of rituximab in the management of patients with acquired thrombotic thrombocytopenic purpura

Wendy Lim, Sara K. Vesely and James N. George

Data supplements

Article Figures & Data


  • Figure 1

    Literature search data. *One article included 2 case series: one case series of patients receiving initial treatment with rituximab and another case series of asymptomatic patients receiving rituximab prophylaxis.


  • Table 1
  • Table 2

    Rituximab for the treatment of patients with TTP and for treatment of ADAMTS13 deficiency during remission: levels of evidence and interpretation

    IndicationKey citationGrade of recommendation and evidence*Interpretation
    Initial treatment of TTPScully, 201182CWe suggest rituximab be considered for this indication. Rituximab may decrease the time to achieve remission and may delay subsequent relapse.
    Treatment of refractory episodes of TTPFroissart, 2012171CWe recommend rituximab be considered for this indication. Patients with refractory TTP require treatment in addition to PEX and conventional corticosteroid regimens, and rituximab appears to be effective.
    Treatment of severe ADAMTS13 deficiency during clinical remissionHie, 2014251CWe recommend against the use of rituximab for this indication. The benefit for relapse-free survival is marginal (P = .049). Patients in the rituximab group received multiple different treatments. The benefit of a single course of rituximab is not known. The natural history of ADAMTS13 activity following recovery from acquired TTP is not known. High-quality evidence is required before treatment of patients with no clinical evidence of TTP can be recommended.
    • * Grade 1 represents a strong recommendation; grade 2 represents a weak recommendation; and grade C represents the lowest quality of evidence.