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. |
| 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.