Treatment and Outcome of Primary and Secondary Thrombotic Microangiopathies

Eşkazan, Ahmet Emre; Salihoğlu, Ayşe

doi:10.1159/000437002

Cited by 5 publications

(3 citation statements)

References 9 publications

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“…Apart from primary thrombotic microangiopathies [32,33], plasma exchange may also be beneficial in other syndromes associated with thrombocytopenia and vWF-ADAMTS13 imbalance [8,[37][38][39][40].…”

Section: Discussionmentioning

confidence: 99%

Plasma von Willebrand factor levels predict in-hospital survival in patients with acute-on-chronic liver failure

Prasanna

Goel

Amirtharaj

et al. 2016

Indian J Gastroenterol

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Section: Discussionmentioning

confidence: 99%

Plasma von Willebrand factor levels predict in-hospital survival in patients with acute-on-chronic liver failure

Prasanna

Goel

Amirtharaj

et al. 2016

Indian J Gastroenterol

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“…Las MAT primarias pueden ser hereditarias o adquiridas, y se presentan de manera espontánea, sin una causa subyacente aparente. Las principales incluyen la PTT debida a deficiencia de ADAMTS13, el SHU asociado a toxina Shiga de Escherichia coli enterohemorrágica y la MAT mediada por complemento (también denominado SHU atípico), que se describen a continuación [3,4,10,11].…”

Section: Mat Primariasunclassified

Microangiopatías trombóticas primarias: una revisión narrativa

Henao-Piedrahita

2021

Med. Lab.

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La microangiopatía trombótica (MAT) es un síndrome donde hay formación de microtrombos en la circulación que llevan a anemia hemolítica microangiopática (AHMA) y trombocitopenia con falla multiorgánica, debido a la isquemia de los tejidos. Las MAT pueden ser primarias sin causa subyacente asociada, como la púrpura trombocitopénica trombótica debida a deficiencia de la enzima ADAMTS13, el síndrome hemolítico urémico debido a la toxina Shiga de Escherichia coli enterohemorrágica, y la MAT producida por alteraciones en la regulación del complemento. Adicionalmente, pueden ser secundarias a enfermedades malignas, infecciosas, metabólicas, autoinmunes o inducidas por el embarazo. Estas patologías requieren diagnóstico y tratamiento oportunos debido a que tienen alta morbimortalidad y se asocian a complicaciones que incluyen enfermedad renal, alteraciones neurológicas como convulsiones, accidente cerebrovascular, coma y muerte. El tratamiento es multidisciplinario y se enfoca en el soporte hemodinámico, transfusional y en el manejo de la etiología cuando esta es identificada. La siguiente revisión pretende explicar de forma clara y precisa los aspectos generales de las MAT primarias.

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“…[13][14][15] Having said that, although ADAMTS13 assays are crucial and help to confirm the diagnosis of iTTP, this is not always straightforward especially in the developing countries, and physicians should not wait for the determination of ADAMTS13 activity to make the diagnosis of iTTP. 16 It may take several days to get the test results, and this could delay the initiation of TPE, which may result in morbidity and/or mortality. Thus, diagnostic work-up should be planned as TPE is started in a patient with possible iTTP.…”

Section: Laboratory Testsmentioning

confidence: 99%

<p>Assessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes</p>

Küçükyurt

Eşkazan

2020

JBM

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Background: Acquired or immune-mediated TTP (iTTP) is a life-threatening thrombotic microangiopathy, characterized by the presence of microangiopathic hemolytic anemia and severe thrombocytopenia, and a variable degree of ischemic organ damage, related to a severe deficiency of ADAMTS13, which is a serine metalloprotease necessary for cleavage of large vWF multimers. There has been a dramatic decrease in mortality rates with the recognition of the pathophysiology of iTTP over the years. Although therapeutic plasma exchange (TPE) together with corticosteroids are the backbone of the upfront treatment of patients with iTTP with successful outcomes, patients may remain refractory and/or relapse following an initial response to this treatment. Methods: We performed a review regarding the pathogenesis, diagnosis, treatment strategies, monitoring, and prognosis of iTTP. Results: There are several new treatment strategies, which can be used among these patients, helping in improving outcomes of iTTP. Rituximab has been shown to be a safe and effective adjunct to TPE, especially in patients with refractory and/or relapse as well as it is increasingly used preemptively to prevent exacerbation or recurrence. Recently, caplacizumab, a nanobody targeting vWF, was approved as an addition to the current regimen of TPE and immunomodulation for patients of iTTP. Conclusion: Specific predictors of relapse in patients in remission can be relevant for an optimal patient management. Different models including ADAMTS13 biomarkers can provide a new screening strategy to identify patients who may predict outcomes and the risk of relapse, benefit from preemptive therapy prior to relapse.

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Treatment and Outcome of Primary and Secondary Thrombotic Microangiopathies

Cited by 5 publications

References 9 publications

Plasma von Willebrand factor levels predict in-hospital survival in patients with acute-on-chronic liver failure

Plasma von Willebrand factor levels predict in-hospital survival in patients with acute-on-chronic liver failure

Microangiopatías trombóticas primarias: una revisión narrativa

<p>Assessment and Monitoring of Patients with Immune-Mediated Thrombotic Thrombocytopenic Purpura (iTTP): Strategies to Improve Outcomes</p>

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