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Published in: Journal of Neurology 10/2023

01-07-2023 | Thrombotic Thrombocytopenic Purpura | Original Communication

Predictors of acute ischemic cerebral lesions in immune-mediated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome

Authors: Lisa Neuman, Adrien Joseph, Raïda Bouzid, Mickael Lescroart, Eric Mariotte, Stéphane Ederhy, Sophie Tuffet, Jean-Luc Baudel, Ygal Benhamou, Lionel Galicier, Steven Grangé, François Provôt, Antoine Neel, Frédéric Pène, Yahsou Delmas, Claire Presne, Pascale Poullin, Alain Wynckel, Pierre Perez, Christelle Barbet, Jean-Michel Halimi, Valérie Chatelet, Jean-Michel Rebibou, Mario Ojeda-Uribe, Cécile Vigneau, Laurent Mesnard, Agnès Veyradier, Elie Azoulay, Paul Coppo, Hugues Chabriat, the participants to the Prospective Multicenter MATRISK study

Published in: Journal of Neurology | Issue 10/2023

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Abstract

Background

The immune form of thrombotic thrombocytopenic purpura (iTTP) and the hemolytic and uremic syndrome (HUS) are two major forms of thrombotic microangiopathy (TMA). Their treatment has been recently greatly improved. In this new era, both the prevalence and predictors of cerebral lesions occurring during the acute phase of these severe conditions remain poorly known.

Aim

The prevalence and predictors of cerebral lesions appearing during the acute phase of iTTP and Shiga toxin-producing Escherichia coli-HUS or atypical HUS were evaluated in a prospective multicenter study.

Methods

Univariate analysis was performed to report the main differences between patients with iTTP and those with HUS or between patients with acute cerebral lesions and the others. Multivariable logistic regression analysis was used to identify the potential predictors of these lesions.

Results

Among 73 TMA cases (mean age 46.9 ± 16 years (range 21–87 years) with iTTP (n = 57) or HUS (n = 16), one-third presented with acute ischemic cerebral lesions on magnetic resonance imagery (MRI); two individuals also had hemorrhagic lesions. One in ten patients had acute ischemic lesions without any neurological symptom. The neurological manifestations did not differ between iTTP and HUS. In multivariable analysis, three factors predicted the occurrence of acute ischemic lesions on cerebral MRI: (1) the presence of old infarcts on cerebral MRI, (2) the level of blood pulse pressure, (3) the diagnosis of iTTP.

Conclusion

At the acute phase of iTTP or HUS, both symptomatic and covert ischemic lesions are detected in one third of cases on MRI. Diagnosis of iTTP and the presence of old infarcts on MRI are associated with the occurrence of such acute lesions as well as increased blood pulse pressure, that may represent a potential target to further improve the therapeutic management of these conditions.
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Metadata
Title
Predictors of acute ischemic cerebral lesions in immune-mediated thrombotic thrombocytopenic purpura and hemolytic uremic syndrome
Authors
Lisa Neuman
Adrien Joseph
Raïda Bouzid
Mickael Lescroart
Eric Mariotte
Stéphane Ederhy
Sophie Tuffet
Jean-Luc Baudel
Ygal Benhamou
Lionel Galicier
Steven Grangé
François Provôt
Antoine Neel
Frédéric Pène
Yahsou Delmas
Claire Presne
Pascale Poullin
Alain Wynckel
Pierre Perez
Christelle Barbet
Jean-Michel Halimi
Valérie Chatelet
Jean-Michel Rebibou
Mario Ojeda-Uribe
Cécile Vigneau
Laurent Mesnard
Agnès Veyradier
Elie Azoulay
Paul Coppo
Hugues Chabriat
the participants to the Prospective Multicenter MATRISK study
Publication date
01-07-2023
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 10/2023
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-023-11830-y

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