Primary Immune Thrombocytopenia: A Translational Research Model for Autoimmune Diseases

Andrès, Emmanuel

doi:10.3390/jcm8111971

Cited by 3 publications

(4 citation statements)

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“…The urgent and optimum therapeutic goal for elderly patients with severe ITP is to increase platelet counts to the safe level and therefore avoid catastrophic bleeding events. Immune abnormalities lead to increased platelet destruction and decreased platelet production in patients with ITP [ 11 , 27 , 28 , 29 ]. Given the high risk of bleeding in elderly patients, it is necessary to find a better treatment option for elderly patients with severe ITP.…”

Section: Discussionmentioning

confidence: 99%

Recombinant Thrombopoietin Effectively Shortens the Time to Response and Increases Platelet Counts in Elderly Patients with Severe Immune Thrombocytopenia

Sun

et al. 2022

JCM

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Background: This study was conducted to investigate the short-term efficacy and safety of rhTPO for the management of severe ITP in the elderly as first-line treatment. Methods: A total of 54 elderly patients with severe ITP were studied, including 39 patients treated with a combination regimen of rhTPO plus standard treatment (glucocorticoid; rhTPO group) and 15 patients treated with glucocorticoid treatment alone (control group). The response rate, time to initial response, peak platelet counts, and time to peak platelet counts were compared, and clinical characteristics correlated with the efficacy of rhTPO were analyzed. The efficacy of rhTPO in the elderly is comparable to the non-elderly in terms of the OR, CR, time to initial response, and peak platelet counts. Results: There were no differences in the overall response (OR) and the complete response (CR) in the rhTPO group compared to the control group. The time to initial response in the rhTPO group was shorter than that in the control group (p = 0.032). In patients without intravenous immunoglobulin (IVIg) and platelet transfusion, the peak platelet counts in the rhTPO group were higher than those in the control group (p = 0.003). Conclusions: Standard glucocorticoid treatment plus rhTPO effectively shortens the time to response and increases platelet counts in the elderly with severe ITP.

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Section: Discussionmentioning

confidence: 99%

Recombinant Thrombopoietin Effectively Shortens the Time to Response and Increases Platelet Counts in Elderly Patients with Severe Immune Thrombocytopenia

Sun

et al. 2022

JCM

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“…Primary immune thrombocytopenia (ITP) is an acquired autoimmune disorder. Numerous studies have highlighted the key role of T cells in the pathogenesis of ITP [ 1 , 2 ]. The Th1/Th2 imbalance was first detected in no other autoimmune diseases but ITP [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

TCR CDR3 Sequencing as a Clue to Elucidate the Landscape of Dysimmunity in Patients with Primary Immune Thrombocytopenia

Zhan

et al. 2022

JCM

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Background: Primary immune thrombocytopenia (ITP) is an autoimmune disorder. The existence of autoreactive T cells has long been proposed in ITP. Yet the identification of autoreactive T cells has not been achieved, which is an important step to elucidate the pathogenesis of ITP. Methods: ITP patients’ peripheral blood was collected prior to the treatment and one month after initiating dexamethasone treatment per related therapeutic guideline. Serum cytokines were profiled to examine T cell subtypes imbalance using a protein chip. TCR Vβ analysis in CD8+T cells of ITP patients, and TCR CDR3 DNA sequencing of CD4+T and CD8+T cells were performed to determine the autoreactive T cells’ clones. Results: Cytokine profiling revealed imbalanced distribution of T cells subtypes, which was confirmed by CD4+T and CD8+T cells’ oligoclonal expansion of TCR Vβ analysis and TCR CDR3 DNA sequencing. VDJ segments were found to be more frequently presented in ITP patients, when compared with health controls. There was an individualized CD4+T cell or CD8+T cell CDR3 sequence in each ITP patient. Conclusions: The present study revealed that T cell clones expanded in ITP patients’ peripheral blood, and each clone had an individualized TCR CDR3 sequence. The expanded T cell clones preferred to use some specific VDJ segment. Further studies are warranted to get access to individualized treatment such as Car-T in patients with ITP.

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“…Immune thrombocytopenia (ITP) is an acquired autoimmune disorder characterized by a decreased platelet count in peripheral blood and increased destruction of platelets and megakaryocytes in peripheral blood and bone marrow, respectively [ 1 ]. The incidence of ITP is estimated to be 100 cases per million years and increases with age [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…These complications include lymphoproliferative disorders (mainly chronic lymphoblastic leukemia), autoimmune disorders such as lupus erythematosus (SLE), infection with different viruses such as cytomegalovirus (CMV), Epstein-Barr virus (EBV), human immunodeficiency virus (HIV), hepatitis C virus (HCV), and infection with the bacterium Helicobacter pylori ( H. pylori ) [ 7 ]. Although the pathogenesis of ITP remains ambiguous, one can assume that due to impaired immunological tolerance following viral infection, immune cells recognize and attack platelet glycoproteins (GPs) (mainly GP IIb/IIIa and Ib/IX) as viral immunogens as a result of molecular mimicry, which ultimately results (including T lymphocytes) in the destruction of platelets [ 1 , 6 , 8 , 9 ]. In parallel to the direct destruction of platelets by T lymphocytes, the immune system can produce autoantibodies against platelets.…”

Section: Introductionmentioning

confidence: 99%

Effect of Helicobacter Pylori eradication on patients with ITP: a meta-analysis of studies conducted in the Middle East

et al. 2021

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Immune thrombocytopenia (ITP) is a bleeding disorder. Helicobacter pylori is a Gram-negative bacterium that is presumed to be associated with ITP and therapeutic response of patients. To evaluate the effect of H. pylori eradication on the platelet count of patients with ITP. To this end, we analyzed studies conducted on the association between H. pylori infection and response to therapy in patients with ITP in Western Asia, with a particular focus on the Middle East region. A systematic search of databases (PubMed/Medline, ISI Web of Science, Cochrane Central) and Google Scholar search engine results was conducted up until January 2020. The keywords included in the search were Helicobacter pylori and/or H. pylori, ITP and/or immune thrombocytopenia. Seven studies comprising a total of 228 H. pyloriinfected patients (193 with successful eradication) were included in this study. The association between H. pylori eradication and ITP was expressed as odds ratios (ORs) and 95% confidence intervals (CI). The findings showed that patients who received eradication treatment for H. pylori infection had significantly higher ORs (OR, 8.83; 95% CI, 2.03-38.35; P=0.004) than those in the non-eradicated group. Our results indicate a significant therapeutic effect of H. pylori eradication on the platelet count of patients with chronic ITP. Given the inherent limitations of this study, including the small number of patients, further studies with more patients are recommended.

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Primary Immune Thrombocytopenia: A Translational Research Model for Autoimmune Diseases

Abstract: Primary immune thrombocytopenia (ITP), formally known as idiopathic thrombocytopenic purpura, is a multifactorial autoimmune disease that is both idiopathic (cause unknown) and rare [...]

Cited by 3 publications

References 10 publications

Recombinant Thrombopoietin Effectively Shortens the Time to Response and Increases Platelet Counts in Elderly Patients with Severe Immune Thrombocytopenia

Recombinant Thrombopoietin Effectively Shortens the Time to Response and Increases Platelet Counts in Elderly Patients with Severe Immune Thrombocytopenia

TCR CDR3 Sequencing as a Clue to Elucidate the Landscape of Dysimmunity in Patients with Primary Immune Thrombocytopenia

Effect of Helicobacter Pylori eradication on patients with ITP: a meta-analysis of studies conducted in the Middle East

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