Recent advances in the mechanisms and treatment of immune thrombocytopenia
Summary: Primary immune thrombocytopenia is an autoimmune disease associated with a reduced peripheral blood platelet count. The phenotype is variable with some patients suffering no bleeding whilst others have severe bleeding which may be fatal. Variability in clinical behaviour and treatment respo...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-02-01
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| Series: | EBioMedicine |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2352396422000093 |
| _version_ | 1818975818350592000 |
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| author | Drew Provan John W. Semple |
| author_facet | Drew Provan John W. Semple |
| author_sort | Drew Provan |
| collection | DOAJ |
| description | Summary: Primary immune thrombocytopenia is an autoimmune disease associated with a reduced peripheral blood platelet count. The phenotype is variable with some patients suffering no bleeding whilst others have severe bleeding which may be fatal. Variability in clinical behaviour and treatment responses reflects its complex underlying pathophysiology. Historically the management has relied heavily on immune suppression. Recent studies have shown that the older empirical immune suppressants fail to alter the natural history of the disease and are associated with a poor quality of life for patients. Newer treatments, such as the thrombopoietin receptor agonists, have transformed ITP care. They have high efficacy, are well tolerated and improve patients’ quality of life. A greater understanding of the underlying pathophysiology of this disorder has helped develop a number of new targeted therapies. These include inhibitors of the neonatal Fc receptor inhibitors, Bruton tyrosine kinase and complement pathway. Here we discuss the mechanisms underlying ITP and the new approach to ITP care. |
| first_indexed | 2024-12-20T16:02:00Z |
| format | Article |
| id | doaj.art-6afc3d9a645e4413bba06c0fa63c0f8d |
| institution | Directory Open Access Journal |
| issn | 2352-3964 |
| language | English |
| last_indexed | 2024-12-20T16:02:00Z |
| publishDate | 2022-02-01 |
| publisher | Elsevier |
| record_format | Article |
| series | EBioMedicine |
| spelling | doaj.art-6afc3d9a645e4413bba06c0fa63c0f8d2022-12-21T19:34:16ZengElsevierEBioMedicine2352-39642022-02-0176103820Recent advances in the mechanisms and treatment of immune thrombocytopeniaDrew Provan0John W. Semple1Centre for Immunology, Blizard Institute, Barts and The London School of Medicine & Dentistry, Queen Mary University of London, London UK; Corresponding author: Dr Drew Provan, Centre for Immunology, Blizard Institute, Barts and The London School of Medicine & Dentistry, Queen Mary University of London, London UK.Division of Hematology and Transfusion Medicine, Lund University, Lund, Sweden; Clinical Immunology and Transfusion Medicine, Office of Medical Services, Region Skåne, Lund, Sweden; Department of Pharmacology and Toxicology, University of Toronto, Toronto, ON, Canada; Professor John W. Semple, Division of Hematology and Transfusion Medicine, Lund University, BMC C14, Klinikgatan 26, 221 84, Lund, Sweden.Summary: Primary immune thrombocytopenia is an autoimmune disease associated with a reduced peripheral blood platelet count. The phenotype is variable with some patients suffering no bleeding whilst others have severe bleeding which may be fatal. Variability in clinical behaviour and treatment responses reflects its complex underlying pathophysiology. Historically the management has relied heavily on immune suppression. Recent studies have shown that the older empirical immune suppressants fail to alter the natural history of the disease and are associated with a poor quality of life for patients. Newer treatments, such as the thrombopoietin receptor agonists, have transformed ITP care. They have high efficacy, are well tolerated and improve patients’ quality of life. A greater understanding of the underlying pathophysiology of this disorder has helped develop a number of new targeted therapies. These include inhibitors of the neonatal Fc receptor inhibitors, Bruton tyrosine kinase and complement pathway. Here we discuss the mechanisms underlying ITP and the new approach to ITP care.http://www.sciencedirect.com/science/article/pii/S2352396422000093immune thrombocytopeniaimmune suppressionthrombopoietin receptor agonists |
| spellingShingle | Drew Provan John W. Semple Recent advances in the mechanisms and treatment of immune thrombocytopenia EBioMedicine immune thrombocytopenia immune suppression thrombopoietin receptor agonists |
| title | Recent advances in the mechanisms and treatment of immune thrombocytopenia |
| title_full | Recent advances in the mechanisms and treatment of immune thrombocytopenia |
| title_fullStr | Recent advances in the mechanisms and treatment of immune thrombocytopenia |
| title_full_unstemmed | Recent advances in the mechanisms and treatment of immune thrombocytopenia |
| title_short | Recent advances in the mechanisms and treatment of immune thrombocytopenia |
| title_sort | recent advances in the mechanisms and treatment of immune thrombocytopenia |
| topic | immune thrombocytopenia immune suppression thrombopoietin receptor agonists |
| url | http://www.sciencedirect.com/science/article/pii/S2352396422000093 |
| work_keys_str_mv | AT drewprovan recentadvancesinthemechanismsandtreatmentofimmunethrombocytopenia AT johnwsemple recentadvancesinthemechanismsandtreatmentofimmunethrombocytopenia |