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The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy (TMA) related to a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. This deficiency is often immune-mediated (iTTP) and related to the presence of anti-ADAMTS13...

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Autores principales: Joseph, Adrien, Joly, Bérangère S., Picod, Adrien, Veyradier, Agnès, Coppo, Paul
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10179719/
https://www.ncbi.nlm.nih.gov/pubmed/37176509
http://dx.doi.org/10.3390/jcm12093068
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author Joseph, Adrien
Joly, Bérangère S.
Picod, Adrien
Veyradier, Agnès
Coppo, Paul
author_facet Joseph, Adrien
Joly, Bérangère S.
Picod, Adrien
Veyradier, Agnès
Coppo, Paul
author_sort Joseph, Adrien
collection PubMed
description Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy (TMA) related to a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. This deficiency is often immune-mediated (iTTP) and related to the presence of anti-ADAMTS13 autoantibodies that enhance its clearance or inhibit its VWF processing activity. iTTP management may be challenging at extreme ages of life. International cohorts of people with TTP report delayed diagnoses and misdiagnoses in children and elderly people. Child-onset iTTP shares many features with adult-onset iTTP: a female predominance, an idiopathic presentation, and the presence of neurological disorders and therapeutic strategies. Long-term follow-ups and a transition from childhood to adulthood are crucial to preventing iTTP relapses, in order to identify the occurrence of other autoimmune disorders and psychosocial sequelae. In contrast, older iTTP patients have an atypical clinical presentation, with delirium, an atypical neurological presentation, and severe renal and cardiac damages. They also have a poorer response to treatment and prognosis. Long-term sequelae are highly prevalent in older patients. Prediction scores for iTTP diagnoses are not used for children and have a lower sensitivity and specificity in patients over 60 years old. ADAMTS13 remains the unique biological marker that is able to definitely confirm or rule out the diagnosis of iTTP and predict relapses during follow-ups.
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spelling pubmed-101797192023-05-13 The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review Joseph, Adrien Joly, Bérangère S. Picod, Adrien Veyradier, Agnès Coppo, Paul J Clin Med Review Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy (TMA) related to a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. This deficiency is often immune-mediated (iTTP) and related to the presence of anti-ADAMTS13 autoantibodies that enhance its clearance or inhibit its VWF processing activity. iTTP management may be challenging at extreme ages of life. International cohorts of people with TTP report delayed diagnoses and misdiagnoses in children and elderly people. Child-onset iTTP shares many features with adult-onset iTTP: a female predominance, an idiopathic presentation, and the presence of neurological disorders and therapeutic strategies. Long-term follow-ups and a transition from childhood to adulthood are crucial to preventing iTTP relapses, in order to identify the occurrence of other autoimmune disorders and psychosocial sequelae. In contrast, older iTTP patients have an atypical clinical presentation, with delirium, an atypical neurological presentation, and severe renal and cardiac damages. They also have a poorer response to treatment and prognosis. Long-term sequelae are highly prevalent in older patients. Prediction scores for iTTP diagnoses are not used for children and have a lower sensitivity and specificity in patients over 60 years old. ADAMTS13 remains the unique biological marker that is able to definitely confirm or rule out the diagnosis of iTTP and predict relapses during follow-ups. MDPI 2023-04-23 /pmc/articles/PMC10179719/ /pubmed/37176509 http://dx.doi.org/10.3390/jcm12093068 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Joseph, Adrien
Joly, Bérangère S.
Picod, Adrien
Veyradier, Agnès
Coppo, Paul
The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review
title The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review
title_full The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review
title_fullStr The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review
title_full_unstemmed The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review
title_short The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review
title_sort specificities of thrombotic thrombocytopenic purpura at extreme ages: a narrative review
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10179719/
https://www.ncbi.nlm.nih.gov/pubmed/37176509
http://dx.doi.org/10.3390/jcm12093068
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