Characteristics and Outcomes of Patients With Cerebral Venous Sinus Thrombosis in SARS-CoV-2 Vaccine–Induced Immune Thrombotic Thrombocytopenia
Authors: Mayte Sánchez van Kammen, Diana Aguiar de Sousa, Sven Poli, Charlotte Cordonnier, Mirjam R. Heldner, Anita van de Munckhof, Katarzyna Krzywicka, Thijs van Haaps, Alfonso Ciccone, Saskia Middeldorp, Marcel M. Levi, Johanna A. Kremer Hovinga, Suzanne Silvis, Sini Hiltunen, Maryam Mansour, Antonio Arauz, Miguel A. Barboza, Thalia S. Field, Georgios Tsivgoulis, Simon Nagel, Erik Lindgren, Turgut Tatlisumak, Katarina Jood, Jukka Putaala, Jose M. Ferro, Marcel Arnold, Jonathan M. Coutinho, Aarti R. Sharma, Ahmed Elkady, Alberto Negro, Albrecht Günther, Alexander Gutschalk, Silvia Schönenberger, Alina Buture, Sean Murphy, Ana Paiva Nunes, Andreas Tiede, Anemon Puthuppallil Philip, Annerose Mengel, Antonio Medina, Åslög Hellström Vogel, Audrey Tawa, Avinash Aujayeb, Barbara Casolla, Brian Buck, Carla Zanferrari, Carlos Garcia-Esperon, Caroline Vayne, Catherine Legault, Christian Pfrepper, Clement Tracol, Cristina Soriano, Daniel Guisado-Alonso, David Bougon, Domenico S. Zimatore, Dominik Michalski, Dylan Blacquiere, Elias Johansson, Elisa Cuadrado-Godia, Emmanuel De Maistre, Emmanuel Carrera, Fabrice Vuillier, Fabrice Bonneville, Fabrizio Giammello, Felix J. Bode, Julian Zimmerman, Florindo d’Onofrio, Francesco Grillo, Francois Cotton, François Caparros, Laurent Puy, Frank Maier, Giosue Gulli, Giovanni Frisullo, Gregory Polkinghorne, Guillaume Franchineau, Hakan Cangür, Hans Katzberg, Igor Sibon, Irem Baharoglu, Jaskiran Brar, Jean-François Payen, Jim Burrow, João Fernandes, Judith Schouten, Katharina Althaus, Katia Garambois, Laurent Derex, Lisa Humbertjean, Lucia Lebrato Hernandez, Lukas Kellermair, Mar Morin Martin, Marco Petruzzellis, Maria Cotelli, Marie-Cécile Dubois, Marta Carvalho, Matthias Wittstock, Miguel Miranda, Mona Skjelland, Monica Bandettini di Poggio, Moritz J. Scholz, Nicolas Raposo, Robert Kahnis, Nyika Kruyt, Olivier Huet, Pankaj Sharma, Paolo Candelaresi, Peggy Reiner, Ricardo Vieira, Roberto Acampora, Rolf Kern, Ronen Leker, Shelagh Coutts, Simerpreet Bal, Shyam S. Sharma, Sophie Susen, Thomas Cox, Thomas Geeraerts, Thomas Gattringer, Thorsten Bartsch, Timothy J. Kleinig, Vanessa Dizonno, Yıldız Arslan
Journal: JAMA Neurology (2021)

<h3>Importance</h3> Thrombosis with thrombocytopenia syndrome (TTS) has been reported after vaccination with the SARS-CoV-2 vaccines ChAdOx1 nCov-19 (Oxford–AstraZeneca) and Ad26.COV2.S (Janssen/Johnson &amp; Johnson). <h3>Objective</h3> To describe the clinical characteristics and outcome of patients with cerebral venous sinus thrombosis (CVST) after SARS-CoV-2 vaccination with and without TTS. <h3>Design, Setting, and Participants</h3> This cohort study used data from an international registry of consecutive patients with CVST within 28 days of SARS-CoV-2 vaccination included between March 29 and June 18, 2021, from 81 hospitals in 19 countries. For reference, data from patients with CVST between 2015 and 2018 were derived from an existing international registry. Clinical characteristics and mortality rate were described for adults with (1) CVST in the setting of SARS-CoV-2 vaccine–induced immune thrombotic thrombocytopenia, (2) CVST after SARS-CoV-2 vaccination not fulling criteria for TTS, and (3) CVST unrelated to SARS-CoV-2 vaccination. <h3>Exposures</h3> Patients were classified as having TTS if they had new-onset thrombocytopenia without recent exposure to heparin, in accordance with the Brighton Collaboration interim criteria. <h3>Main Outcomes and Measures</h3> Clinical characteristics and mortality rate. <h3>Results</h3> Of 116 patients with postvaccination CVST, 78 (67.2%) had TTS, of whom 76 had been vaccinated with ChAdOx1 nCov-19; 38 (32.8%) had no indication of TTS. The control group included 207 patients with CVST before the COVID-19 pandemic. A total of 63 of 78 (81%), 30 of 38 (79%), and 145 of 207 (70.0%) patients, respectively, were female, and the mean (SD) age was 45 (14), 55 (20), and 42 (16) years, respectively. Concomitant thromboembolism occurred in 25 of 70 patients (36%) in the TTS group, 2 of 35 (6%) in the no TTS group, and 10 of 206 (4.9%) in the control group, and in-hospital mortality rates were 47% (36 of 76; 95% CI, 37-58), 5% (2 of 37; 95% CI, 1-18), and 3.9% (8 of 207; 95% CI, 2.0-7.4), respectively. The mortality rate was 61% (14 of 23) among patients in the TTS group diagnosed before the condition garnered attention in the scientific community and 42% (22 of 53) among patients diagnosed later. <h3>Conclusions and Relevance</h3> In this cohort study of patients with CVST, a distinct clinical profile and high mortality rate was observed in patients meeting criteria for TTS after SARS-CoV-2 vaccination.