Acquired Thrombotic Thrombocytopenic Purpura After BNT162b2 COVID-19 Vaccine: Case Report and Literature Review

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy that is deadly if not treated promptly. The treatment of choice in patients presenting with TTP is plasma exchanges. However, immunosuppressive therapy and caplacizumab have significantly improved outcomes in TTP. This microangiopathy is classically divided into 2 entities: hereditary and acquired TTP (aTTP), caused by an autoantibody against ADAMTS 13. We present a case study of a patient wth TTP occurring after a second dose of the BNT162b2 (Pfizer-BioNTech) COVID-19 vaccine along with a review of the literature. A 55-year-old patient presented with gastrointestinal symptoms, anemia, and severe thrombocytopenia. The blood film revealed the presence of schistocytes. A diagnosis of aTTP was established because the patient had severe ADAMTS 13 deficiency and autoantibodies against ADAMTS 13 were positive. This episode occurred 10 days after the patient received the COVID-19 vaccine. The patient received plasma exchanges, prednisone, rituximab, and caplacizumab and achieved complete remission. Ten patients with aTTP induced by the COVID-19 vaccine have been reported in the literature. Most of these situations occurred after the second dose of COVID-19 vaccine, and 7 patients were noted to have received the BNT162b2 vaccine. Caplacizumab was used in 6 patients, and complete remission was achieved in 8 patients.

COVID-19, post-acute COVID-19 syndrome (PACS, “long COVID”) and post-COVID-19 vaccination syndrome (PCVS, “post-COVIDvac-syndrome”): Similarities and differences

“…Autoantibodies were detected during the acute phase of COVID-19 [316] , [317] , [318] , [319] , [320] , [321] , [322] , [323] , [324] , [325] , [326] , [327] , [328] , [329] , [330] , [331] , [332] , [333] , months afterwards (predicting PACS symptoms) [334] , [335] , in PACS [336] , [337] , [338] , [339] , [340] , and in PCVS [295] , [298] , [341] , [342] , [343] , [344] , [345] , [346] , [347] , [348] , [349] , [350] , [351] , [352] , [353] , [354] , [355] , [356] , [357] , [358] , [359] , [360] , [361] , [362] , [363] , [364] , [365] . In the absence of PCVS after COVID-19 vaccination, autoantibodies are generally not present [366] , [367] , [368] .…”

New‐onset and relapsed thrombotic microangiopathy post‐COVID‐19 vaccination

“…A total of 84 cases of TMA associated with COVID‐19 vaccination were found in 58 articles, 2,9–65 including 64 patients diagnosed with TTP (Table 1), 17 cases manifested as aHUS (Table 2, cases 1–17), and three patients presented as unclassified TMA (Table 2, cases 18–20). Subsequently, relevant clinical records were extracted for analysis, including baseline characteristics, laboratory tests, treatment options, and outcomes.…”

Coronavirus disease 2019 (COVID‐19): Focus on peripheral blood cell morphology

“…In addition, schistocytes and TTP have even been observed after anti‐COVID‐19 vaccination. 63 , 97 , 98 , 99 , 100 , 101 …”

“…A moderate increase of schistocytes (2%–4%) was reported in 50 consecutive patients using the Sysmex XN‐9000 blood cell counter with microscope confirmation, in association with increased lactate dehydrogenase and D‐dimer and mild decrease of A disintegrin and metalloproteinase with thrombospondin type 1 motifs, member 13 (ADAMTS13) activity, 96 a combination that supports the presence of microangiopathy in COVID‐19. In addition, schistocytes and TTP have even been observed after anti‐COVID‐19 vaccination 63,97–101 …”