First and fatal case of autoimmune acquired factor XIII/13 deficiency after COVID‐19/SARS‐CoV‐2 vaccination

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] .…”

Autoimmune Acquired Factor XIII/13 Deficiency after SARS-CoV-2 mRNA Vaccination

“…However, other autoimmune bleeding disorder, acquired hemophilia A, was reported post vaccination. 7 8 9 10 AiF13D post-SARS-CoV-2 vaccination is extremely rare except for another case 11 ; more clinical experiences are warranted in the future.…”

Frequent bleeding symptoms associated with autoimmune acquired factor XIII/13 deficiency due to anti‐factor XIII A and B subunit antibodies

“…On the other hand, Kotake et al reported a case of AiF13D with anti‐F13‐A antibody in which the F13 activity continued to be low despite treatment with rituximab, cyclophosphamide, and F13 concentrates, and eventually died of cerebral hemorrhage after 3 years 7 . Recently, Shimoyama et al reported a case of AiF13D with anti‐F13‐A antibody after COVID‐19/SARS‐CoV‐2 vaccination who died of cerebral and subarachnoid hemorrhage within 1 month after the start of immunosuppressive therapy and F13 replacement therapy 8 . It should be noted that the risk of intracranial hemorrhage is high in cases where the F13 activity is markedly reduced in both congenital and acquired F13 deficiency 1,9 .…”

“…7 Recently, Shimoyama et al reported a case of AiF13D with anti-F13-A antibody after COVID-19/SARS-CoV-2 vaccination who died of cerebral and subarachnoid hemorrhage within 1 month after the start of immunosuppressive therapy and F13 replacement therapy. 8 It should be noted that the risk of intracranial hemorrhage is high in cases where the F13 activity is markedly reduced in both congenital and acquired F13 deficiency. 1,9 Long-term antibody eradication therapy and careful hemostatic management are required until remission is achieved in refractory patients with AiF13D.…”