Hereditary thrombotic thrombocytopenic purpura and COVID-19: Impacts of vaccination and infection in this rare disease

Erika Tarasco; Anne Sophie von Krogh; Radomira Hrdlickova; Thomas R Braschler; Teresa Iwaniec; Paul N Knöbl; Eriko Hamada; Oleg Pikovsky; Stefan Farese; Odit Gutwein; Petr Kessler; Nina H Schultz; Charis von Auer; Jerzy Windyga; Kenneth Friedman; Ingrid Hrachovinova; James N George; Masanori Matsumoto; Reinhard Schneppenheim; Bernhard Lämmle; Johanna Anna Kremer Hovinga

doi:10.1002/rth2.12814

Hereditary thrombotic thrombocytopenic purpura and COVID-19: Impacts of vaccination and infection in this rare disease

Res Pract Thromb Haemost. 2022 Oct 21;6(7):e12814. doi: 10.1002/rth2.12814. eCollection 2022 Oct.

Authors

Erika Tarasco^{1

2}, Anne Sophie von Krogh^{3

4}, Radomira Hrdlickova⁵, Thomas R Braschler⁶, Teresa Iwaniec⁷, Paul N Knöbl⁸, Eriko Hamada⁹, Oleg Pikovsky¹⁰, Stefan Farese¹¹, Odit Gutwein¹², Petr Kessler¹³, Nina H Schultz¹⁴, Charis von Auer¹⁵, Jerzy Windyga¹⁶, Kenneth Friedman¹⁷, Ingrid Hrachovinova¹⁸, James N George¹⁹, Masanori Matsumoto⁹, Reinhard Schneppenheim²⁰, Bernhard Lämmle^{1

21}, Johanna Anna Kremer Hovinga^{1

2}

Affiliations

¹ Department of Hematology and Central Hematology Laboratory Inselspital, Bern University Hospital Bern Switzerland.
² Department for BioMedical Research (DBMR) University of Bern Bern Switzerland.
³ Department of Hematology St. Olavs Hospital, Trondheim University Hospital Trondheim Norway.
⁴ Department of Clinical and Molecular Medicine, Faculty of Medicine and Health Sciences Norwegian University of Science and Technology Trondheim Norway.
⁵ Blood Center University Hospital Ostrava Ostrava Czech Republic.
⁶ Center for Clinical Hematology Luzerne Kantonsspital Lucerne Switzerland.
⁷ Department of Hematology Jagiellonian University Medical College Krakow Poland.
⁸ Division of Hematology and Hemostasis, Department of Medicine I Medical University of Vienna Vienna Austria.
⁹ Department of Blood Transfusion Medicine Nara Medical University Kashihara Japan.
¹⁰ Transfusion Medicine Institute, Faculty of Health Sciences, Ben-Gurion University of the Negev Soroka University Medical Center Beer-Sheva Israel.
¹¹ Department of Nephrology Burgerspital Solothurn Switzerland.
¹² Department of Hematology Shamir Medical Center Zerifin Israel.
¹³ Department of Hematology and Transfusion Medicine Hospital Pelhrimov Pelhrimov Czech Republic.
¹⁴ Department of Hematology Oslo University Hospital Oslo Norway.
¹⁵ Department of Hematology, Oncology, and Pneumology, Center for Thrombosis and Hemostasis University Medical Center Mainz Mainz Germany.
¹⁶ Department of Haemostasis Disorders and Internal Medicine Institute of Hematology and Transfusion Medicine Warsaw Poland.
¹⁷ Division of Hematology and Oncology, Versiti Blood Center of Wisconsin Medical College of Wisconsin Milwaukee Wisconsin USA.
¹⁸ Institute of Hematology and Blood Transfusion Prague Czech Republic.
¹⁹ Department of Biostatistics & Epidemiology College of Public Health, University of Oklahoma Health Sciences Center Oklahoma City Oklahoma USA.
²⁰ Department of Pediatric Hematology and Oncology University Medical Center Hamburg-Eppendorf Hamburg Germany.
²¹ Center for Thrombosis and Hemostasis University Medical Center Mainz Mainz Germany.

Abstract

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.

Keywords: COVID‐19; SARS‐CoV‐2; congenital thrombotic thrombocytopenic purpura (cTTP); hereditary thrombotic thrombocytopenic purpura (hTTP); vaccines.