Immune-Mediated Thrombotic Thrombocytopenic Purpura Following mRNA-Based COVID-19 Vaccine BNT162b2: Case Report and Mini-Review of the Literature.

Buetler, Vanessa Alexandra; Agbariah, Nada; Schild, Deborah Pia; Liechti, Fabian D; Wieland, Anna; Andina, Nicola; Hammann, Felix; Kremer Hovinga, Johanna A (2022). Immune-Mediated Thrombotic Thrombocytopenic Purpura Following mRNA-Based COVID-19 Vaccine BNT162b2: Case Report and Mini-Review of the Literature. Frontiers in medicine, 9, p. 890661. Frontiers 10.3389/fmed.2022.890661

fmed-09-890661.pdf - Published Version
Available under License Creative Commons: Attribution (CC-BY).

Download (406kB) | Preview


An increasing number of case reports have associated vaccinations against coronavirus disease 2019 (COVID-19) with immune-mediated thrombotic thrombocytopenic purpura (iTTP), a very rare but potentially life-threatening thrombotic microangiopathy, which leads to ischemic organ dysfunction. Thrombus formation in iTTP is related to a severe deficiency of the specific von Willebrand-factor-cleaving protease ADAMTS13 due to ADAMTS13 autoantibodies.


We present a case of iTTP following exposure to the mRNA-based COVID-19 vaccine BNT162b2 (Comirnaty®, Pfizer-BioNTech). In addition, we review previously reported cases in the literature and assess current evidence.


Apart from our case, twenty cases of iTTP occurring after COVID-19 vaccination had been published until the end of November 2021. There were 11 male and 10 female cases; their median age at diagnosis was 50 years (range 14-84 years). Five patients (24%) had a preexisting history of iTTP. Recombinant adenoviral vector-based vaccines were involved in 19%, mRNA-based vaccines in 81%. The median onset of symptoms after vaccination was 12 days (range 5-37), with 20 cases presenting within 30 days. Treatment included therapeutic plasma exchange in all patients. Additional rituximab, caplacizumab, or both these treatments were given in 43% (9/21), 14% (3/21), and 24% (5/21) of cases, respectively. One patient died, despite a prolonged clinical course in one patient, all surviving patients were in clinical remission at the end of the observational period.


Clinical features of iTTP following COVID-19 vaccination were in line with those of pre-pandemic iTTP. When timely initiated, an excellent response to standard treatment was seen in all cases. ADAMTS13 activity should be determined pre-vaccination in patients with a history of a previous iTTP episode. None of the reported cases met the WHO criteria for assessing an adverse event following immunization (AEFI) as a consistent causal association to immunization. Further surveillance of safety data and additional case-based assessment are needed.

Item Type:

Journal Article (Further Contribution)


04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory
04 Faculty of Medicine > Department of General Internal Medicine (DAIM) > Clinic of General Internal Medicine
04 Faculty of Medicine > Department of Cardiovascular Disorders (DHGE) > Clinic of Cardiology

UniBE Contributor:

Bütler, Vanessa Alexandra; Agbariah, Nada; Schild, Deborah; Liechti, Fabian; Wieland-Greguare-Sander, Anna; Andina, Nicola; Hammann, Felix and Kremer Hovinga Strebel, Johanna Anna


600 Technology > 610 Medicine & health








Pubmed Import

Date Deposited:

08 Jun 2022 08:28

Last Modified:

05 Dec 2022 16:20

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

ADAMTS13 COVID-19 vaccine mRNA SARS-CoV-2 vaccine purpura thrombotic thrombocytopenic




Actions (login required)

Edit item Edit item
Provide Feedback