Determining the Functional Effect of COVID-19 on Adametase 13 Protease activity in Patients with TTP (Thrombotic Thrombocytopenic Purpura): A Systematic Review
Determining the Functional Effect of COVID-19 on Adametase 13 Protease activity in Patients with TTP (Thrombotic Thrombocytopenic Purpura): A Systematic Review
Elnaz Azadi,1Ali Ahmadi,2,*Dariush D. Farhud,3
1. MD. Student, Department of Medical Sciences, Kermanshah University of Medical Sciences, Qasr Shirin, Kermanshah, Iran 2. BSc. Student, Department of Biological Sciences and Technologies, Islamic Azad University Sari Branch, Sari, Iran 3. School of Public Health, Tehran University of Medical Sciences, Tehran, Iran
Introduction: Thrombocytopenic purpura (TTP) is a blood condition that affects 3 to 10 adults per million population in one year and is deficient in (<10%) ADAMTS13 (a di-integrin and metalloproteinase), a protein. The cut is defined for the phone. Thrombotic microangiopathies are a group of disorders that are mainly related to endothelial dysfunction. These endothelial disorders are caused by several imbalances between platelets, the endothelium, the immune system, and the production of cytokines. With the advent of Covid-19 vaccines, the severity of hospitalization of patients and the progression of Covid-19 disease in the acute condition is greatly reduced. Autoimmune hematologic complications such as vaccine-induced immune thrombocytopenia (VITT), immune thrombocytopenic purpura (ITP), and TTP have also been reported after COVID-19 vaccination. The pathophysiology of TTP de-Novo remains unclear after vaccination with two schools of thought. First, it expresses undetected latent TTP, which is shown by patients after a stimulus (vaccine) with the onset of symptoms within a few days of receiving the vaccine. Second, it increases the potential for the formation of autoantibodies against ADAMTS13, which it presents through molecular mimicry mechanisms. In addition, there is evidence that ADAMTS13 deficiency alone is not sufficient for acute relapse in patients with acquired or recurrent TTP. There is a need for a "second shock" in the form of infection or inflammation for acute TTP deposition, in which case the COVID-19 vaccine was considered. (Thrombotic Thrombocytopenic Purpura).
Methods: This is a secondary study (Systematic Review - 2022) looking for preferred case reports for systematic reviews and meta-analysis recommendations (PRISMA) that we searched in the PubMed, EMBASE, and EBSCO databases for published studies on TTP. There were no restrictions based on language, age, or country of origin. The first search was conducted on May 1, 2022, followed by an additional search on May 12, 2022. The two authors independently screened all search results from three databases at the title and abstract level, and if any, the discrepancies were resolved. By discussion or judgment by the third author. We retrieved all available resources in the studies provided for additional resources. The following keywords were used to identify the reports: "COVID-19" [Mesh] AND "TTP [Mesh]" OR "Purpura" [Mesh] AND "ADAMTS13 Protein" [Mesh] AND "COVID-19 Vaccines"
Results: Normally in the body, proteases called Adams to break down 13 von Willebrand multimeters, and in its absence, these multimers remain, causing platelet binding and aggregation. This deficiency is inherited or more commonly acquired as a result of the production of antibodies against Adams 13 and if its activity level reaches less than 10%, thrombotic thrombocytopenic purpura disease develops and its five symptoms include fever, Thrombocytopenia is microangiopathic hemolytic anemia, renal failure, and neurological symptoms. In its peripheral blood smear, there are red blood cells in the form of schistocytes. The enzyme is lactate dehydrogenase and its diagnosis is based on clinical evaluation of thrombocytopenia and microangiopathic hemolytic anemia. Assay for the activity of Adams 13 is not widely available. Thrombotic thrombocytopenic mortality is relatively significant, reaching about 10% at 18 months after starting steroid treatment and plasma replacement.
Conclusion: Evaluation of thrombocytopenia after vaccination poses an important diagnostic dilemma, and physicians should consider the likelihood of TTP given the associated mortality and the need for appropriate treatment, as the risk of TTP mortality without early treatment is -80. 90% remains high.