Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura

Novel therapies in thrombotic thrombocytopenic purpura

“…A retrospective study of 24 patients with relapsing TTP showed a decrease in the relapse rate from 0.74 relapses/patient‐year to 0.1 relapses/patient‐year after splenectomy after a median follow up of 115 months 50. It could also be argued that the long‐term remission rates that have been reported after splenectomy are comparable to those seen with rituximab 51, 52. The complications related to splenectomy are minimal, but are increased when splenectomy is used for refractory TTP.…”

“…Three different groups have evaluated its use as addition to PEX and steroids in the acute episode of TTP 5, 8, 52. All three of them reported fewer and later relapses in the rituximab treated patients.…”

Adjuvant rituximab to prevent TTP relapse

“…1 Although their disease was more intractable at initial presentation, patients in the rituximab group had a significantly lower rate of relapse than the standard therapy group during followup (P 5 .009). Two of 16 patients (13%) in the rituximab group relapsed at 2.5 and 9.9 years, whereas 9 of 21 patients (43%) in the standard therapy group relapsed at a median of 3.1 years (range 0.4-5.9 years).…”

“…Their data raise the question of whether all or selected patients with a first episode of acquired TTP should be treated with adjuvant rituximab to prevent recurrence. 1 A cquired TTP is a thrombotic microangiopathy attributable to autoantibody-mediated deficiency of the von Willebrand factor-cleaving protease, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Standard therapy with plasma exchange (PEX), supplemented with corticosteroids, is lifesaving.…”

“…Other studies have also demonstrated reduced relapse rates with rituximab, although these investigations were limited by inclusion of both de novo and relapsed patients, use of historical rather than contemporaneous nonrituximab-treated controls, and variable follow-up. [3][4][5] Although the duration of follow-up was not uniform among patients in the current study, 1 it was similar between treatment groups, permitting estimation of risk reduction and number needed to treat. As shown in the table, rituximab was associated with an estimated For personal use only.…”