COVID 19 infection associated with thrombotic thrombocytopenic purpura

Dhingra, Gaurav; Maji, Manideepa; Mandal, Saikat; Vaniyath, Sudeep; Negi, Gita; Nath, Uttam Kumar

doi:10.1007/s11239-021-02452-0

Cited by 18 publications

(18 citation statements)

References 15 publications

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“…This VWF/ADAMTS13 imbalance may support the concept of a secondary TMA‐like syndrome potentially present in some critically ill COVID‐19 patients and contributing to the microthrombi formation in pulmonary alveolar capillaries 8,11,15,19 . Surprisingly, however, ~20 TTP cases likely triggered by a SARS‐CoV‐2 infection were recently reported 20–25 . Also, the link between a TMA process and the COVID‐19‐associated microthrombosis may be supported by the clinical improvement and the reduction of circulating thromboinflammatory markers (i.e., the VWF/ADAMTS13 ratio) induced by plasma exchange undertaken in some critically ill COVID‐19 patients 15,26 .…”

Section: Introductionmentioning

confidence: 72%

“…8,11,15,19 Surprisingly, however, ~20 TTP cases likely triggered by a SARS-CoV-2 infection were recently reported. [20][21][22][23][24][25] Also, the link between a TMA process and the COVID-19-associated microthrombosis may be supported by the clinical improvement and the reduction of circulating thromboinflammatory markers (i.e., the VWF/ADAMTS13 ratio) induced by plasma exchange undertaken in some critically ill COVID-19 patients. 15,26 One ex vivo study suggested that purified recombinant ADAMTS13 should be considered as a potential therapy for COVID-19 patients.…”

Section: Introductionmentioning

confidence: 99%

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Imbalance of von Willebrand factor and ADAMTS13 axis is rather a biomarker of strong inflammation and endothelial damage than a cause of thrombotic process in critically ill COVID‐19 patients

Joly

Darmon

Dekimpe

et al. 2021

Journal of Thrombosis and Haemostasis

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Background: Critically ill patients with coronavirus disease 2019 (COVID-19) are prone to developing macrothrombosis and microthrombosis. COVID-19 has been reported to be rarely associated with thrombotic microangiopathies. A disintegrin and metalloprotease with thrombospondin type I repeats, member 13 (ADAMTS13) severe deficiency, the hallmark of thrombotic thrombocytopenic purpura (TTP), induces the formation of platelet, unusually large von Willebrand factor (VWF) multimer microthrombi. In immune-mediated TTP, ADAMTS13 adopts specifically an open conformation. The VWF/ADAMTS13 couple may contribute to the microthrombi formation in pulmonary alveolar capillaries in COVID-19. Objective: To investigate clinical features, hemostatic laboratory parameters, VWF/ ADAMTS13 axis, and ADAMTS13 conformation in critically ill COVID-19 patients at admission. Methods: Fifty three critically ill COVID-19 patients were enrolled between March 18 and May 9 2020 in a monocentric hospital. Results:The median age was 59 years and the male-to-female ratio was 2.8/1. We reported seven pulmonary embolisms and 15 deaths. Biological investigations showed increased fibrinogen and factor V levels, and strongly increased D-dimers correlated with mortality. No patient presented severe thrombocytopenia nor microangiopathic hemolytic anemia. An imbalance between high VWF antigen levels and normal or slightly decreased ADAMTS13 activity levels (strongly elevated VWF/ADAMTS13 ratio) was correlated with mortality. Three patients had a partial quantitative defi-How to cite this article: Joly BS, Darmon M, Dekimpe C, et al. Imbalanced of von Willebrand factor and ADAMTS13 axis is rather a biomarker of strong inflammation and endothelial damage than a cause of thrombotic process in critically ill COVID-19 patients.

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Section: Introductionmentioning

confidence: 72%

Section: Introductionmentioning

confidence: 99%

Imbalance of von Willebrand factor and ADAMTS13 axis is rather a biomarker of strong inflammation and endothelial damage than a cause of thrombotic process in critically ill COVID‐19 patients

Joly

Darmon

Dekimpe

et al. 2021

Journal of Thrombosis and Haemostasis

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“…SARS-CoV2 has been reported as a possible trigger for autoimmune and inflammatory dysregulation 38 including i-TTP, which has been described as a further dramatic life-threatening clinical presentation of SARS-Cov-2 infection. [39][40][41] There have been several case reports describing the development of i-TTP secondary to COVID-19 infection in adults 39 and only one pediatric case. 42 The majority of these cases had i-TTP concurrently with COVID-19 infection, but i-TTP has also been described after one month from SARS-Cov2 infection.…”

Section: Discussionmentioning

confidence: 99%

“…While the majority of i‐TTP cases are of unknown ethology, secondary i‐TTP can be associated to certain autoimmune disorders like systemic lupus erythematosus, malignancies, medications, as well as viral infections like HIV, 36 CMV, 37 and SARS‐CoV‐2. SARS‐CoV2 has been reported as a possible trigger for autoimmune and inflammatory dysregulation 38 including i‐TTP, which has been described as a further dramatic life‐threatening clinical presentation of SARS‐Cov‐2 infection 39–41 . There have been several case reports describing the development of i‐TTP secondary to COVID‐19 infection in adults 39 and only one pediatric case 42 .…”

Section: Discussionmentioning

confidence: 99%

Immune‐mediated thrombotic thrombocytopenic purpura in a Jehovah’s Witness – Effectiveness of incorporating extracorporeal immunoadsorption to caplacizumab, steroids and rituximab

Cardesa-Salzmann

Stephan

Simon

et al. 2022

Clinical Case Reports

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“…Commentaries, review articles and abstracts without full texts were excluded as well. We included 8 articles including the data of 11 patients with COVID-19 associated TTP [5][6][7][8][9][10][11][12] as depicted in PRISMA flow diagram (Figure 1).…”

Section: Study Selection and Characteristicsmentioning

confidence: 99%

COVID-19 Associated Thrombotic Thrombocytopenic Purpura: A Case Report and Systematic Review

Chaudhary¹,

Nasir²,

Syed³

et al. 2022

Preprint

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Introduction: The proliferation of literature regarding COVID-19 pandemic has served to highlight a wide spectrum of disease manifestations and complications like thrombotic microangiopathies. Our review with a brief case presentation highlights the increasing recognition of TTP in COVID-19 and describes its salient characteristics. Methods: We screened the available literature in Pubmed, EMBASE and Cochrane database from inception till April 2022 of articles mentioning COVID-19 associated TTP in English Language. Results: From 404 records, we included 8 articles mentioning data of 11 patients in our review. TTP was predominantly reported in females (72%) with a mean age of 48.2 years (SD 15.1). Dyspnea was the most common symptom in 1/3rd of patients (36.6%). Neurological symptoms were reported in 27.3% of cases. The time to diagnosis of TTP was 10 days (SD: 5.8) from onset of Covid-19. All 11 cases underwent plasma exchange (PLEX), with a mean of 12 sessions per patient, whereas six cases received Rituximab (54.5%), and three received Caplacizumab (27.3%). One patient died from the illness. Conclusion: This review of available literature highlights the atypical and refractory nature of COVID-19 associated TTP. It required longer sessions of PLEX with half of the patients receiving at least one immunosuppressant.

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COVID 19 infection associated with thrombotic thrombocytopenic purpura

Cited by 18 publications

References 15 publications

Imbalance of von Willebrand factor and ADAMTS13 axis is rather a biomarker of strong inflammation and endothelial damage than a cause of thrombotic process in critically ill COVID‐19 patients

Imbalance of von Willebrand factor and ADAMTS13 axis is rather a biomarker of strong inflammation and endothelial damage than a cause of thrombotic process in critically ill COVID‐19 patients

Immune‐mediated thrombotic thrombocytopenic purpura in a Jehovah’s Witness – Effectiveness of incorporating extracorporeal immunoadsorption to caplacizumab, steroids and rituximab

COVID-19 Associated Thrombotic Thrombocytopenic Purpura: A Case Report and Systematic Review

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