Efficacy and safety of rituximab in adult patients with idiopathic relapsing or refractory thrombotic thrombocytopenic purpura: Results of a Spanish multicenter study

Rubia, Javier de la; Moscardó, Federico; Gómez, Marı́a; Guàrdia, Ramón; Rodríguez, P.; Sebrango, Ana; Zamora, Concepción; Debén, Guillermo; Goterris, Rosa; Lopez, R. Rodriguez; Peña, Francisco Ayala de la; Pujol, M; Vidaller, Antonio; Río-Garma, Julio del; Sanz, Miguel Á.

doi:10.1016/j.transci.2010.09.018

Cited by 55 publications

(21 citation statements)

References 25 publications

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“…Initial data showed, and confirmed in individual cases, small series and larger cohorts (relative to the disease incidence), the benefit of rituximab in relapsing and refractory TTP (Chemnitz et al , ; Gutterman et al , ; Zheng et al , ; Ahmad et al , ; Sallah et al , ; Fakhouri et al , ; Reddy et al , ; Scully et al , ; Jasti et al , ; Ling et al , ; de la Rubia et al , ). Indeed, funding restrictions are such that, in some areas, this remains the point at which rituximab is used.…”

Section: Treatmentmentioning

confidence: 86%

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

2014

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SummaryThrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life‐threatening thrombotic microangiopathies that require rapid diagnosis and treatment. They are defined by microangiopathic haemolytic anaemia and thrombocytopenia, with renal involvement primarily in aHUS and neurological and cardiological sequelae in TTP. Prompt treatment for most cases of both conditions is with plasma exchange initially and monoclonal therapy (rituximab in TTP and eculizumab in aHUS) as the mainstay of therapy. Here we discuss the diagnosis and therapy for both disorders.

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Section: Treatmentmentioning

confidence: 86%

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

2014

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“…Despite some advances there is a considerable mortality for patients that either relapse or are considered refractory and there remains a pressing need to evaluate novel agents in this clinical setting (Bell et al , ; Rock et al , ; Shumak et al , ; Bandarenko & Brecher, ; Clark et al , ; Willis & Bandarenko, ; Forzley et al , ; Levandovsky et al , ; Scully et al , ; Kremer Hovinga et al , ). Several reports as well as two systematic reviews have previously evaluated rituximab as an effective treatment option for relapsing and refractory TTP patients (Zheng et al , ; Jasti et al , ; Ling et al , ; Elliott et al , ; De la Rubia et al , ; Tun & Villani, ; Canadian Apheresis Group [unpublished observations]; Scully et al , ). Scully et al () recently reported the benefit of up‐front rituximab used in combination with therapeutic plasma exchange in 40 newly diagnosed TTP [based on ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) testing] patients; in this study the addition of rituximab led to a reduction in relapses from 20–50% to 10% compared with historical registry‐based controls.…”

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confidence: 99%

A phase‐II sequential case‐series study of all patients presenting to four plasma exchange centres with presumed relapsed/refractory thrombotic thrombocytopenic purpura treated with rituximab

et al. 2015

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SummaryThe primary objective of this phase II study was to evaluate the efficacy of rituximab in the management of adult patients with physician-diagnosed presumed thrombotic thrombocytopenic purpura (TTP); relapsed or refractory. We conducted a multicentre study in four Canadian hospital-based apheresis units. Forty patients with presumed TTP (20 refractory and 20 relapsing) were sequentially enrolled and all received rituximab in a standardized manner. A complete response was documented in 14 of 19 refractory patients by week 8 and 15/16 were alive and in remission at 52 weeks (one patient was lost to follow-up, one was a non-responder, and three died). Among relapsing patients, 16/18 had a complete response at week 8 and 18/18 at week 52 (one patient lost to follow-up and one withdrew). At 1 year, all relapsing and 85% of refractory patients survived. Of 38/40 patients who had ADMATS13 testing at study entry, 13/19 refractory and 10/19 relapsing patients had ADAMTS13 < 10% (typical TTP); whereas 6/19 refractory and 9/19 relapsing cases had ADAMTS13 > 10% (other thrombotic microangiopathy; TMA). Refractory-typical TTP in contrast to refractory-other TMA and all relapsing patients treated with plasma exchange and rituximab, were less likely to be responsive and more likely to die or relapse.

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“…The 34 studies from 11 countries reported at least one outcome of interest and followed a total of 1182 patients between the years of 1982 and 2013 (Table ) . The majority of studies were single center; 32% of the studies were conducted at multiple centers.…”

Section: Resultsmentioning

confidence: 99%

Long‐term outcomes of thrombotic microangiopathy treated with plasma exchange: A systematic review

et al. 2016

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With the adoption of plasma exchange as standard treatment for thrombotic microangiopathy (TMA), more patients are surviving and long-term outcomes have greater relevance. We conducted a systematic review to synthesize and evaluate the quality of evidence on long-term outcomes of TMA among adults treated with plasma exchange and to identify factors that may be associated with a worse long-term prognosis. We searched databases from 1980 to 2013 for eligible articles published in any language. We included studies that reported outcomes in at least ten adults with a history of TMA treated with plasma exchange and at least 6 months of follow-up. We abstracted data in duplicate and assessed the methodological quality of each study using an assessment tool developed based on recommended validity criteria. We screened 6672 articles, reviewed 213, and included 34 studies totaling 1182 patients (study median [range], 24 ). The mean (or median) follow-up ranged from 6 months to 13 years. The cumulative incidence of relapse and mortality was highly variable and ranged from 3 to 84 and 0 to 61%, respectively. The incidence of other outcomes across 10 studies also varied (outcomes included hypertension, kidney disease, preeclampsia, stroke, seizure, severe cognitive impairment, and depression); in three other studies, long-term neurocognitive function and health-related quality of life were significantly lower than in the general population. Patients who survive an episode of TMA may be susceptible to long-term vascular complications, but the magnitude of this risk and how to mitigate it remains unclear.

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Efficacy and safety of rituximab in adult patients with idiopathic relapsing or refractory thrombotic thrombocytopenic purpura: Results of a Spanish multicenter study

Cited by 55 publications

References 25 publications

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

A phase‐II sequential case‐series study of all patients presenting to four plasma exchange centres with presumed relapsed/refractory thrombotic thrombocytopenic purpura treated with rituximab

Long‐term outcomes of thrombotic microangiopathy treated with plasma exchange: A systematic review

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