Cardiovascular disease is a leading cause of mortality among TTP survivors in clinical remission

Sukumar, Senthil; Brodsky, Max A.; Hussain, Sarah; Yanek, Lisa R.; Moliterno, Alison R.; Cataland, Spero R.; Chaturvedi, Shruti

doi:10.1182/bloodadvances.2020004169

Cited by 31 publications

(32 citation statements)

References 28 publications

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“…Age has negative impacts both in the short term (atypical neurological presentation and delayed diagnosis), but also in the long term, as older survivors show increased long‐term mortality that increases for every 10 years of age range 108 . Besides iTTP‐related deaths, cardiovascular complications are prevalent in iTTP survivors, and are the leading primary cause of death (~28%), followed by malignancy (~21%) and infection (~14%), while other/unknown causes represent 10% of cases 109 . The incidence of strokes in iTTP survivors is fivefold higher than in the general population, and is associated with lower ADAMTS13 activity during remission 110 .…”

Section: Milestones In Ttp Treatment: Meet the New Playersmentioning

confidence: 99%

TTP: From empiricism for an enigmatic disease to targeted molecular therapies

et al. 2022

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The 100th anniversary of the first description of Thrombotic Thrombocytopenic Purpura (TTP) as a disease by Dr. Eli Moschcowitz approaches. For many decades, TTP remained mostly a mysterious fatal condition, where diagnosis was often postmortem. Initially a pentad of symptoms was identified, a pattern that later revealed to be fallible. Sporadic observations led to empiric interventions that allowed for the first impactful breakthrough in TTP treatment, almost 70 years after its first description: the introduction of plasma exchange and infusions as treatments. The main body of knowledge within the field was gathered in the latest three decades: patient registries were set and proved crucial for advancements; the general mechanisms of disease have been described; the diagnosis was refined; new treatments and biomarkers with improvements on prognosis and management were introduced.Further changes and improvements are expected in the upcoming decades. In this review, we provide a brief historic overview of TTP, as an illustrative example of the success of translational medicine enabling to rapidly shift from a management largely based on empiricism to targeted therapies and personalized medicine, for the benefit of patients. Current management options and present and future perspectives in this still evolving field are summarized.

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Section: Milestones In Ttp Treatment: Meet the New Playersmentioning

confidence: 99%

TTP: From empiricism for an enigmatic disease to targeted molecular therapies

et al. 2022

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“…HUS are associated with an increased long-term risk of stroke and myocardial infarction. Interestingly, another subset of TMA patients with TTP also seems to have high longterm cardio-vascular risk as well [87][88][89]. This cardiovascular risk could be increased in patients with subsequent sequelae of acute kidney injury.…”

Section: What Is the Impact Of Complement Dysregulation In Hus Patients Care?mentioning

confidence: 99%

Complement Blockade Is a Promising Therapeutic Approach in a Subset of Critically Ill Adult Patients with Complement-Mediated Hemolytic Uremic Syndromes

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Guillotin

et al. 2022

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Thrombotic microangiopathy (TMA) gathers consumptive thrombocytopenia, mechanical haemolytic anemia, and organ damage. Hemolytic uremic syndromes (HUS) are historically classified as primary or secondary to another disease once thrombotic thrombocytopenic purpura (TTP), Shiga-toxin HUS, and cobalamin C-related HUS have been ruled out. Complement genetics studies reinforced the link between complement dysregulation and primary HUS, contributing to reclassifying some pregnancy- and/or post-partum-associated HUS and to revealing complement involvement in severe and/or refractory hypertensive emergencies. By contrast, no firm evidence allows a plausible association to be drawn between complement dysregulation and Shiga-toxin HUS or other secondary HUS. Nevertheless, rare complement gene variants are prevalent in healthy individuals, thus providing an indication that an investigation into complement dysregulation should be carefully balanced and that the results should be cautiously interpreted with the help of a trained geneticist. Several authors have suggested reclassifying HUS in two entities, regardless of they are complement-mediated or not, since the use of eculizumab, an anti-C5 antibody, dramatically lowers the proportion of patients who die or suffer from end-stage renal disease within the year following diagnosis. Safety and the ideal timing of eculizumab discontinuation is currently under investigation, and the long-term consequences of HUS should be closely monitored over time once patients exit emergency departments.

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“…1,2 Patients with acquired autoimmune TTP (iTTP) also have a high risk for stroke and other thrombotic events during remission, especially when plasma ADAMTS13 activity is less than normal. 3,4 Current management of hTTP and of iTTP during remission is not sufficient for prevention of stroke and other thrombotic events. [1][2][3][4] The result is premature deaths among patients with hTTP 1 or iTTP.…”

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confidence: 99%

“…3,4 Current management of hTTP and of iTTP during remission is not sufficient for prevention of stroke and other thrombotic events. [1][2][3][4] The result is premature deaths among patients with hTTP 1 or iTTP. 4 Although low-dose aspirin is effective for secondary prevention of stroke and other thrombotic events, it has been assumed that aspirin may not be effective for preventing stroke and thrombotic events in patients with TTP.…”

mentioning

confidence: 99%

“…[1][2][3][4] The result is premature deaths among patients with hTTP 1 or iTTP. 4 Although low-dose aspirin is effective for secondary prevention of stroke and other thrombotic events, it has been assumed that aspirin may not be effective for preventing stroke and thrombotic events in patients with TTP. Aspirin is effective because it prevents platelet aggregation by blocking the activation of the platelet fibrinogen receptor, αIIbβIII.…”

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confidence: 99%

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