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

• DOI: 10.1007/s00415-023-11830-y
• Publication Date: 2023-07-01T04:05:11Z
• 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; ; Augusto Jean-Fran...; Azoulay Elie; Barbay Virginie; Benhamou Ygal; Charasse Christophe; Charvet-Rumpler Anne; Chauveau Dominique; Ribes Davis; Choukroun Gabriel; Coindre Jean-Phil...; Coppo Paul; Delmas Yahsou; Kwon Theresa; Salanoubat Célia; Dossier Antoine; Fain Olivier; Ville Simon; Frémeaux-Bacchi V...; Galicier Lionel; Grangé Steven; Guidet Bertrand; Halimi Jean-Michel; Hamidou Mohamed; Neel Antoine; Fornecker Luc-Mat...; Hié Miguel; Jacobs Frédéric; Joly Bérangère; Kanouni Tarik; Kaplanski Gilles; Rieu Claire; Le Guern Véronique; Moulin Bruno; Rebibou Jean-Michel; Ojeda Uribe Mario; Parquet Nathalie; Pène Frédéric; Perez Pierre; Poullin Pascale; Marie Manon; Presne Claire; Provôt François; Mesnard Laurent; Saheb Samir; Seguin Amélie; Servais Aude; Stépanian Alain; Veyradier Agnès; Vigneau Cécile; Wynckel Alain; Zunic Patricia
• Keywords: Adult; Aged, 80 and over; Complications; Purpura, Thrombotic Thrombocytopenic; 610; Thrombosis; Middle Aged; Stroke; Young Adult; Thrombotic thrombocytopenic purpura; Autonomic Nervous System Diseases; Infarction; 616; Hemolytic-Uremic Syndrome; Hemolytic uremic syndrome; Humans; Prospective Studies; [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology; Acute infarcts; MRI; Aged

AbstractBackgroundThe 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.AimThe prevalence and predictors of cerebral lesions appearing during the acute phase of iTTP and shigatoxin-producingEscherichia coli-HUS or atypical HUS were evaluated in a prospective multicenter study.MethodsUnivariate 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.ResultsAmong 73 TMA cases (mean age 46.89 ± 15.99 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, 3 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.ConclusionCerebral MRI is crucial to detect both symptomatic and covert ischemic lesions at the acute phase of iTTP and HUS and helps identify patients with old infarcts, at the highest risk of neurological worsening. The diagnosis of iTTP further increases the risk of ischemic lesions but also an increased level of blood pressure that may represent a potential target to further improve the therapeutic management of these conditions.Key points-One third of patients with immune-mediated thrombotic thrombocytopenic purpura (iTTP) or the hemolytic uremic syndrome (HUS) present with acute ischemic cerebral lesions on magnetic resonance imagery (MRI);-The cerebral manifestations on MRI did not differ between iTTP and HUS;-The presence of old infarcts on cerebral MRI, the level of blood pulse pressure and a diagnosis of iTTP predict the occurrence of acute ischemic lesions on cerebral MRI.