Relapse of immune thrombotic thrombocytopenic purpura (iTTP) possibly triggered by COVID-19 vaccination and/or concurrent COVID-19 infection

Tse, Brandon; Pavenski, Katerina

doi:10.1136/bcr-2021-247524

Cited by 5 publications

(5 citation statements)

References 28 publications

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“…We assumed that the relapse of acute iTTP was facilitated by the patient's persistent severe ADAMTS13 deficiency and was triggered by her advanced pregnancy and SARS-CoV-2 infection. 1,12 Daily TPE and high-dose glucocorticoids were initiated resulting in clinical remission (Figure 1A). At that time, obstetrical examination (including an ultrasound as well as a cardiotocogram) demonstrated a healthy foetus with normal intrauterine growth as well as no maternal signs for pre-eclampsia.…”

Section: Case De Scr Ip Tionmentioning

confidence: 99%

“…At presentation, ADAMTS13 activity in plasma was severely deficient (<5%), and a high‐titre ADAMTS13 inhibitor was detected. We assumed that the relapse of acute iTTP was facilitated by the patient's persistent severe ADAMTS13 deficiency and was triggered by her advanced pregnancy and SARS‐CoV‐2 infection 1,12 . Daily TPE and high‐dose glucocorticoids were initiated resulting in clinical remission (Figure 1A).…”

Section: Case Descriptionmentioning

confidence: 99%

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Successful management of refractory immune‐mediated thrombotic thrombocytopenic purpura during pregnancy and delivery using the anti‐VWF nanobody caplacizumab

Schimmer,

Sutter,

Bachofner

et al. 2024

Br J Haematol

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SummaryPregnancy is a potential trigger of acute thrombotic thrombocytopenic purpura (TTP). The management of pregnancy‐associated immune‐mediated TTP (iTTP) can be challenging, especially when it is refractory to standard treatment. Caplacizumab, a nanobody to von Willebrand factor (VWF) blocking its A1 domain, is a valuable new therapeutic option. Its use is, however, not approved during pregnancy and breastfeeding. We describe the successful off‐label administration of caplacizumab during pregnancy and delivery in a patient with refractory iTTP. The favourable outcome without significant thrombotic or haemorrhagic complications indicates that caplacizumab may be an effective and safe treatment option in refractory iTTP during pregnancy.

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Section: Case De Scr Ip Tionmentioning

confidence: 99%

Section: Case Descriptionmentioning

confidence: 99%

Successful management of refractory immune‐mediated thrombotic thrombocytopenic purpura during pregnancy and delivery using the anti‐VWF nanobody caplacizumab

Schimmer,

Sutter,

Bachofner

et al. 2024

Br J Haematol

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“…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.…”

Section: Clinical Characteristics Of Tma Patients Associated With Cov...mentioning

confidence: 99%

“…There were 64 affected individuals presented as TTP (Table 1), 2,9–49 including 44 newly diagnosed (68.8%) and 20 relapsed (31.3%). The median age was 49.5 (14–84) years.…”

Section: Clinical Characteristics Of Tma Patients Associated With Cov...mentioning

confidence: 99%

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

2023

Journal of Medical Virology

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Thrombotic microangiopathy (TMA) associated with coronavirus disease 2019 (COVID-19) vaccination has been reported, however, the clinical characteristics and pathogenesis remained mysterious. We reviewed 84 TMA cases post-COVID-19 vaccination, including 64 patients diagnosed with thrombotic thrombocytopenic purpura (TTP), 17 cases presented as atypical hemolytic uremic syndrome (aHUS), and three cases manifested as unclassified TMA. TMA episodes were mostly associated with messenger RNA vaccines. For TTP, 67.6% of females developed symptoms after the first dose of the vaccine, and 63.0% of males were secondary to the second dose (p = 0.015). Compared with TTP, aHUS generally appeared within 7 days (p = 0.002) and showed higher levels of serum creatinine (p < 0.001). 87.5% of TTP received plasma exchange (PEX)-based treatment, and 52.9% of aHUS adopted non-PEX-based therapies (p < 0.001). Mechanistically, complement dysfunction, neutrophil activation, and the generation of pathogenic autoantibodies resulting from molecular mimicry contribute to explaining the pathogenesis of TMA post-COVID-19 vaccination.

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“…Similarly, it is likely that in COVID-19, as in other infectious diseases, molecular mimicry, a hyperactive immune system, and the virus itself represents the triad of plausible causal auto-immune events [ 91 , 98 ]. Other auto-immune conditions related to hemostasis and associated with COVID-19 include ITP [ 99 , 100 , 101 ] and TTP [ 102 , 103 ]. In terms of the latter, a large body of data indicates that COVID-19 is often associated with ADAMTS13 deficiency [ 104 , 105 , 106 ]; however, this may often be due to exhaustion of ADAMTS13 reserves due to the associated elevated release of VWF, rather than any immune basis or TTP.…”

Section: Autoantibodies Interfering With Hemostasis In Covid-19 and A...mentioning

confidence: 99%

Autoimmune Diseases Affecting Hemostasis: A Narrative Review

Favaloro

Pasalic

Lippi

2022

IJMS

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Hemostasis reflects a homeostatic mechanism that aims to balance out pro-coagulant and anti-coagulant forces to maintain blood flow within the circulation. Simplistically, a relative excess of procoagulant forces can lead to thrombosis, and a relative excess of anticoagulant forces can lead to bleeding. There are a wide variety of congenital disorders associated with bleeding or thrombosis. In addition, there exist a vast array of autoimmune diseases that can also lead to either bleeding or thrombosis. For example, autoantibodies generated against clotting factors can lead to bleeding, of which acquired hemophilia A is the most common. As another example, autoimmune-mediated antibodies against phospholipids can generate a prothrombotic milieu in a condition known as antiphospholipid (antibody) syndrome (APS). Moreover, there exist various autoimmunity promoting environments that can lead to a variety of antibodies that affect hemostasis. Coronavirus disease 2019 (COVID-19) represents perhaps the contemporary example of such a state, with potential development of a kaleidoscope of such antibodies that primarily drive thrombosis, but may also lead to bleeding on rarer occasions. We provide here a narrative review to discuss the interaction between various autoimmune diseases and hemostasis.

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Relapse of immune thrombotic thrombocytopenic purpura (iTTP) possibly triggered by COVID-19 vaccination and/or concurrent COVID-19 infection

Cited by 5 publications

References 28 publications

Successful management of refractory immune‐mediated thrombotic thrombocytopenic purpura during pregnancy and delivery using the anti‐VWF nanobody caplacizumab

Successful management of refractory immune‐mediated thrombotic thrombocytopenic purpura during pregnancy and delivery using the anti‐VWF nanobody caplacizumab

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

Autoimmune Diseases Affecting Hemostasis: A Narrative Review

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