Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura

<h4>Background</h4> Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic microangiopathy requiring urgent therapeutic plasma exchange (TPE). However, the exact impact of a slight delay in TPE initiation on the subsequent patients’ outcome is st...

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Autores principales:	Arthur Renaud, Aurélie Caristan, Amélie Seguin, Christian Agard, Gauthier Blonz, Emmanuel Canet, Marion Eveillard, Pascal Godmer, Julie Graveleau, Marie Lecouffe-Desprets, Hervé Maisonneuve, François Perrin, Mohamed Hamidou, Antoine Néel
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/93ec5618b3fd4c6182ed87fcfa769cac
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spelling	oai:doaj.org-article:93ec5618b3fd4c6182ed87fcfa769cac2021-11-25T06:19:26ZDeleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura1932-6203https://doaj.org/article/93ec5618b3fd4c6182ed87fcfa769cac2021-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8604371/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Background</h4> Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic microangiopathy requiring urgent therapeutic plasma exchange (TPE). However, the exact impact of a slight delay in TPE initiation on the subsequent patients’ outcome is still controversial. <h4>Aim</h4> We aimed to study the frequency, short-term neurological consequences, and determinants of diagnostic delay in iTTP. <h4>Methods</h4> We conducted a retrospective monocentric study including patients with a first acute episode of iTTP (2005–2020) classified into 2 groups: delayed (>24h from first hospital visit, group 1) and immediate diagnosis (≤24h, group 2). <h4>Results</h4> Among 42 evaluated patients, 38 were included. Eighteen cases (47%) had a delayed diagnosis (median: 5 days). The main misdiagnosis was immune thrombocytopenia (67%). The mortality rate was 5% (1 death in each group). Neurological events (stroke/TIA, seizure, altered mental status) occurred in 67% vs 30% patients in group 1 and 2, respectively (p = 0.04). Two patients in group 1 exhibited neurological sequelae. The hospital length of stay was longer in group 1 (p = 0.02). At the first hospital evaluation, potential alternative causes of thrombocytopenia were more prevalent in group 1 (33% vs 5%, p = 0.04). Anemia was less frequent in group 1 (67% vs 95%, p = 0.04). All patients had undetectable haptoglobin levels. By contrast, 26% of schistocytes counts were <1%, mostly in group 1 (62% vs 11%, p = 0.01). <h4>Conclusion</h4> Diagnostic delay is highly prevalent in iTTP, with a significant impact on short-term neurological outcome. In patients with profound thrombocytopenia, the thorough search for signs of incipient organ dysfunction, systematic hemolysis workup, and proper interpretation of schistocytes count are the key elements of early diagnosis of TTP.Arthur RenaudAurélie CaristanAmélie SeguinChristian AgardGauthier BlonzEmmanuel CanetMarion EveillardPascal GodmerJulie GraveleauMarie Lecouffe-DespretsHervé MaisonneuveFrançois PerrinMohamed HamidouAntoine NéelPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 11 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Arthur Renaud Aurélie Caristan Amélie Seguin Christian Agard Gauthier Blonz Emmanuel Canet Marion Eveillard Pascal Godmer Julie Graveleau Marie Lecouffe-Desprets Hervé Maisonneuve François Perrin Mohamed Hamidou Antoine Néel Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura
description	<h4>Background</h4> Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare life-threatening thrombotic microangiopathy requiring urgent therapeutic plasma exchange (TPE). However, the exact impact of a slight delay in TPE initiation on the subsequent patients’ outcome is still controversial. <h4>Aim</h4> We aimed to study the frequency, short-term neurological consequences, and determinants of diagnostic delay in iTTP. <h4>Methods</h4> We conducted a retrospective monocentric study including patients with a first acute episode of iTTP (2005–2020) classified into 2 groups: delayed (>24h from first hospital visit, group 1) and immediate diagnosis (≤24h, group 2). <h4>Results</h4> Among 42 evaluated patients, 38 were included. Eighteen cases (47%) had a delayed diagnosis (median: 5 days). The main misdiagnosis was immune thrombocytopenia (67%). The mortality rate was 5% (1 death in each group). Neurological events (stroke/TIA, seizure, altered mental status) occurred in 67% vs 30% patients in group 1 and 2, respectively (p = 0.04). Two patients in group 1 exhibited neurological sequelae. The hospital length of stay was longer in group 1 (p = 0.02). At the first hospital evaluation, potential alternative causes of thrombocytopenia were more prevalent in group 1 (33% vs 5%, p = 0.04). Anemia was less frequent in group 1 (67% vs 95%, p = 0.04). All patients had undetectable haptoglobin levels. By contrast, 26% of schistocytes counts were <1%, mostly in group 1 (62% vs 11%, p = 0.01). <h4>Conclusion</h4> Diagnostic delay is highly prevalent in iTTP, with a significant impact on short-term neurological outcome. In patients with profound thrombocytopenia, the thorough search for signs of incipient organ dysfunction, systematic hemolysis workup, and proper interpretation of schistocytes count are the key elements of early diagnosis of TTP.
format	article
author	Arthur Renaud Aurélie Caristan Amélie Seguin Christian Agard Gauthier Blonz Emmanuel Canet Marion Eveillard Pascal Godmer Julie Graveleau Marie Lecouffe-Desprets Hervé Maisonneuve François Perrin Mohamed Hamidou Antoine Néel
author_facet	Arthur Renaud Aurélie Caristan Amélie Seguin Christian Agard Gauthier Blonz Emmanuel Canet Marion Eveillard Pascal Godmer Julie Graveleau Marie Lecouffe-Desprets Hervé Maisonneuve François Perrin Mohamed Hamidou Antoine Néel
author_sort	Arthur Renaud
title	Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura
title_short	Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura
title_full	Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura
title_fullStr	Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura
title_full_unstemmed	Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura
title_sort	deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura
publisher	Public Library of Science (PLoS)
publishDate	2021
url	https://doaj.org/article/93ec5618b3fd4c6182ed87fcfa769cac
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Deleterious neurological impact of diagnostic delay in immune-mediated thrombotic thrombocytopenic purpura

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