INTRODUCTION: Severe COVID-19 is associated with an important increase of von Willebrand factor and mild lowering of ADAMTS13 activity that may, in the presence of a strong inflammatory reaction, increase the risk of acute thrombotic thrombocytopenic purpura (TTP). Although acute episodes of immune-mediated TTP associated with COVID-19 or SARS-CoV-2 vaccination have been reported, data about clinical evolution of hereditary TTP (hTTP) during the pandemic are scarce. METHOD: We conducted a survey among adult patients of the International Hereditary TTP Registry about SARS-CoV-2 vaccination, COVID-19, and occurrence of acute hTTP episodes. RESULTS: Of 122 adult hTTP patients invited to participate, 86 (70.5%) responded. Sixty-five had been vaccinated (75.6%), of which 14 had received in addition a booster, resulting in 139 individual vaccine shots. Although vaccinations in patients on plasma prophylaxis were done within 1 week of the last plasma infusion, all 23 patients treated with plasma on demand were vaccinated without prior plasma infusions. One patient on uninterrupted weekly plasma infusions presented within 3 days from his second vaccination with neurological symptoms and computed tomography scan 9 days later showed subacute ischemic/hemorrhagic frontal lobe infarction. A second male patient developed acute myocarditis after his second dose of mRNA-1273 vaccine. Twelve (14%) patients had COVID-19, associated with an acute hTTP episode in three of them: one patient had a transient ischemic attack, one a stroke, and a pregnant woman was hospitalized to intensify plasma treatment. DISCUSSION: The risk of an acute episode triggered by COVID-19 seems higher than following vaccination in hTTP patients, who can be safely vaccinated against SARS-CoV-2.

Blood Center University Hospital Ostrava Ostrava Czech Republic

Center for Clinical Hematology Luzerne Kantonsspital Lucerne Switzerland

Center for Thrombosis and Hemostasis University Medical Center Mainz Mainz Germany

Department for BioMedical Research University of Bern Bern Switzerland

Department of Biostatistics and Epidemiology College of Public Health University of Oklahoma Health Sciences Center Oklahoma City Oklahoma USA

Department of Blood Transfusion Medicine Nara Medical University Kashihara Japan

Department of Clinical and Molecular Medicine Faculty of Medicine and Health Sciences Norwegian University of Science and Technology Trondheim Norway

Department of Haemostasis Disorders and Internal Medicine Institute of Hematology and Transfusion Medicine Warsaw Poland

Department of Hematology and Central Hematology Laboratory Inselspital Bern University Hospital Bern Switzerland

Department of Hematology and Transfusion Medicine Hospital Pelhrimov Pelhrimov Czech Republic

Department of Hematology Jagiellonian University Medical College Krakow Poland

Department of Hematology Oncology and Pneumology Center for Thrombosis and Hemostasis University Medical Center Mainz Mainz Germany

Department of Hematology Oslo University Hospital Oslo Norway

Department of Hematology Shamir Medical Center Zerifin Israel

Department of Hematology St Olavs Hospital Trondheim University Hospital Trondheim Norway

Department of Nephrology Burgerspital Solothurn Switzerland

Department of Pediatric Hematology and Oncology University Medical Center Hamburg Eppendorf Hamburg Germany

Division of Hematology and Hemostasis Department of Medicine 1 Medical University of Vienna Vienna Austria

Division of Hematology and Oncology Versiti Blood Center of Wisconsin Medical College of Wisconsin Milwaukee Wisconsin USA

Institute of Hematology and Blood Transfusion Prague Czech Republic

Transfusion Medicine Institute Faculty of Health Sciences Ben Gurion University of the Negev Soroka University Medical Center Beer Sheva Israel

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