Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura

Coppo, Paul; Veyradier, Agnès

doi:10.1016/j.lpm.2011.10.024

Cited by 61 publications

(57 citation statements)

References 88 publications

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“…Of the 2927 patients with TMA included in the national registry, 385 had acquired idiopathic TTP and severe acquired ADAMTS13 deficiency and were treated in the acute phase according to consensual guidelines. [20][21][22] Forty-seven of these patients died during the acute phase of the disease. Among the 338 surviving patients in remission, 105 were not included in this study because ADAMTS13 activity was not systematically assessed during follow-up or because of other missing data, or because the follow-up was shorter than 1 year.…”

Section: Resultsmentioning

confidence: 99%

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Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura

Hié

Galicier

François

et al. 2014

Blood

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153

160

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• Patients with a history of acquired TTP and persistent severe ADAMTS13 deficiency during remission are at high risk of relapse and death.• Preemptive infusions of rituximab in remission significantly decrease TTP relapse rate.In acquired thrombotic thrombocytopenic purpura (TTP), the persistence of severe ADAMTS13 deficiency (<10%) during remission is associated with more relapse. Preemptive (ie, after remission) administration of rituximab in these patients to prevent relapses remains controversial. We performed a cross-sectional analysis of 12-year follow-up data to compare the relapse incidence with or without preemptive rituximab infusion. Among 48 patients who experienced at least one episode of acquired TTP followed by severe ADAMTS13 deficiency during remission, 30 received preemptive rituximab (group 1); the other 18 did not (group 2). After a median of 17 months (interquartile range [IQR], 11-29) following rituximab, the relapse incidence decreased from 0.57 episodes/year (IQR, 0.46-0.7) to 0 episodes/year (IQR, 0-0.81) (P < .01) in group 1. ADAMTS13 activity 3 months after the first rituximab infusion increased to 46% (IQR, 30%-68%). Nine patients required additional courses of rituximab. In 5 patients, ADAMTS13 activity failed to increase durably. Four patients experienced manageable adverse effects. In group 2, the relapse incidence was higher (0.5 relapses/year; IQR, 0.12-0.5; P < .01). Relapse-free survival was longer in group 1 (P 5 .049). A persistent severe ADAMTS13 deficiency during TTP remission should prompt consideration of preemptive rituximab to prevent relapses. (Blood. 2014;124(2):204-210)

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Section: Resultsmentioning

confidence: 99%

“…5,[20][21][22] This protocol was consensually acknowledged nationwide and has been implemented by all participating centers since October 2000. Briefly, therapeutic plasma exchange was carried out daily immediately after TTP diagnosis.…”

Section: Patients and Treatmentmentioning

confidence: 99%

Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura

Hié

Galicier

François

et al. 2014

Blood

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153

160

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“…The importance of ADAMTS13 activity level in TTP is controversial 18,19. Coppo et al showed that 71% of patients clinically diagnosed with TTP had severe ADAMTS13 deficiency 20. In Oklahoma registry data of 301 patients 13% of all patients, and 33% of patients with idiopathic TTP-HUS, had very severe ADAMTS-13 deficiency (<5% activity) 12.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura - analysis of clinical features, laboratory characteristics and therapeutic outcomeof 24 patients treated at a Tertiary Care Center in Saudi Arabia

Iqbal¹,

Zaidi²,

Motabi³

et al. 2016

Pak J Med Sci

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Objective:Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease. The primary aim was overall response rate (ORR) assessment in the treated patientsMethods:This retrospective study included 24 patients treated during 2006-2015. TTP patients with microangiopathic hemolysis (MAHA) and thrombocytopenia were included. We analyzed clinical features, laboratory characteristics and treatment outcomes of 24 TTP patients treated at our tertiary care center (KFMC).Results:Twenty-four TTP patients (18 females; 6 males) had a mean age of 33.5±13.9 years; 22(91%) had neurologic features, 7(29%) fever, 10(42%) renal impairment; 4(20.83%) cardiac manifestations; 22(91.7%) had triad with additional neurologic abnormalities; only 2(8.2%) had pentad of TTP. Majority (54.16%) had idiopathic TTP. All patients received therapeutic plasma exchange (TPE); 23(95.8%) received adjunctive corticosteroids and 13(54.2%) received rituximab either due to refractoriness to TPE on ~day7, or earlier. Twenty-one out of 24 (87.5%) achieved complete remission (CR) without any subsequent relapse. At 22 months (median, range 1-113), 20 patients (83.3%) are alive at the time of report. Three patients died during acute episode because of sever disease or delayed treatment and one died in CR.Conclusion:TPE, steroids and or rituximab was very effective in preventing high risk of mortality and achieving durable CR in 87.5% of patients. More awareness is needed for early diagnosis and early referral to centers with appropriate tertiary care facilities..

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“…In some cases, acquired TTP occurs in association with specific conditions that have to be identified for appropriate management: HIV infection, connective tissue disease, pregnancy, cancer, or treatment with antiplatelet agents. 1 The standard treatment of acquired TTP consists mainly of daily therapeutic plasma exchange (TPE) that allows ADAMTS13 repletion and, to a lesser extent, removal of anti-ADAMTS13 antibodies and possibly pro-aggregatory substances. TPE transformed the historically fatal prognosis of TTP, leading to the current overall survival rates of 80%-85%.…”

Section: Learning Objectivesmentioning

confidence: 99%

Treatment of thrombotic thrombocytopenic purpura beyond therapeutic plasma exchange

Coppo

Froissart

2015

Hematology

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Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of 80%-85%. However, relapses occur in ϳ40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates. In more severe patients, salvage strategies may include twice-daily TPE, pulses of cyclophosphamide, vincristine, as well as splenectomy in more desperate cases.In this life-threatening disease, relapse prevention represents a major goal. Persistent severe acquired ADAMTS13 deficiency in patients who are otherwise in remission is associated with a high risk of relapse and preemptive treatment with rituximab may be considered in this context.In the coming years, the TTP therapeutic landscape should be enriched by original strategies stemming from clinical experience and new agents that are currently being evaluated in large, ideally international, clinical trials. Promising agents under evaluation include N-acetylcysteine, bortezomib, recombinant ADAMTS13, and inhibitors of the glycoprotein-Ib/IX-von Willebrand factor axis. Learning Objectives• Patients with acquired TTP who experience a suboptimal response (refractoriness or disease exacerbation) with standard management need a more intensive treatment • Salvage therapies typically include rituximab, and in the more severe cases twice daily plasma exchange, boluses of cyclophosphamide, vincristine, and splenectomy • A persistent severe acquired ADAMTS13 deficiency during TTP remission should prompt consideration of preemptive rituximab to prevent relapses

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Current management and therapeutical perspectives in thrombotic thrombocytopenic purpura

Cited by 61 publications

References 88 publications

Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura

Preemptive rituximab infusions after remission efficiently prevent relapses in acquired thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura - analysis of clinical features, laboratory characteristics and therapeutic outcomeof 24 patients treated at a Tertiary Care Center in Saudi Arabia

Treatment of thrombotic thrombocytopenic purpura beyond therapeutic plasma exchange

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