Table 2.

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

Indication	Key citation	Grade of recommendation and evidence*	Interpretation
Initial treatment of TTP	Scully, 20118	2C	We 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 TTP	Froissart, 201217	1C	We 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 remission	Hie, 201425	1C	We 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.