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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2023
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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. |
format | Online Article Text |
id | pubmed-10179719 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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