{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T03:21:00Z","timestamp":1785900060937,"version":"3.56.0"},"reference-count":134,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2024,6,26]],"date-time":"2024-06-26T00:00:00Z","timestamp":1719360000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"PDSA Grant (Platelet Disorder Support Association; pdsa.org)","award":["PID2020-117265GB-I00"],"award-info":[{"award-number":["PID2020-117265GB-I00"]}]},{"name":"I + D Excelencia 2020 project grant","award":["PID2020-117265GB-I00"],"award-info":[{"award-number":["PID2020-117265GB-I00"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hematology Reports"],"abstract":"<jats:p>Immune thrombocytopenia (ITP) is an autoimmune disease characterized by an isolated decrease in platelet count and an increased risk of bleeding. The pathogenesis is complex, affecting multiple components of the immune system and causing both peripheral destruction of platelets and inadequate production in the bone marrow. In this article, we review the treatment of ITP from a historical perspective, discussing first line and second line treatments, and management of refractory disease.<\/jats:p>","DOI":"10.3390\/hematolrep16030039","type":"journal-article","created":{"date-parts":[[2024,6,28]],"date-time":"2024-06-28T05:22:38Z","timestamp":1719552158000},"page":"390-412","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Treatment of Immune Thrombocytopenia: Contextualization from a Historical Perspective"],"prefix":"10.3390","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4840-7822","authenticated-orcid":false,"given":"Daniel","family":"Mart\u00ednez-Carballeira","sequence":"first","affiliation":[{"name":"Department of Hematology, Hospital Universitario Central de Asturias (HUCA), 33011 Oviedo, Spain"},{"name":"Platelet Research Lab, Instituto de Investigaci\u00f3n Sanitaria del Principado de Asturias (ISPA), 33011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9711-8037","authenticated-orcid":false,"given":"\u00c1ngel","family":"Bernardo","sequence":"additional","affiliation":[{"name":"Department of Hematology, Hospital Universitario Central de Asturias (HUCA), 33011 Oviedo, Spain"},{"name":"Platelet Research Lab, Instituto de Investigaci\u00f3n Sanitaria del Principado de Asturias (ISPA), 33011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Caro","sequence":"additional","affiliation":[{"name":"Department of Hematology, Hospital Universitario Central de Asturias (HUCA), 33011 Oviedo, Spain"},{"name":"Platelet Research Lab, Instituto de Investigaci\u00f3n Sanitaria del Principado de Asturias (ISPA), 33011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inmaculada","family":"Soto","sequence":"additional","affiliation":[{"name":"Department of Hematology, Hospital Universitario Central de Asturias (HUCA), 33011 Oviedo, Spain"},{"name":"Platelet Research Lab, Instituto de Investigaci\u00f3n Sanitaria del Principado de Asturias (ISPA), 33011 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8443-900X","authenticated-orcid":false,"given":"Laura","family":"Guti\u00e9rrez","sequence":"additional","affiliation":[{"name":"Platelet Research Lab, Instituto de Investigaci\u00f3n Sanitaria del Principado de Asturias (ISPA), 33011 Oviedo, Spain"},{"name":"Department of Medicine, University of Oviedo, 33006 Oviedo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,6,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2386","DOI":"10.1182\/blood-2008-07-162503","article-title":"Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: Report from an international working group","volume":"113","author":"Rodeghiero","year":"2009","journal-title":"Blood"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1002\/ajh.21616","article-title":"The incidence of immune thrombocytopenic purpura in children and adults: A critical review of published reports","volume":"85","author":"Terrell","year":"2010","journal-title":"Am. J. Hematol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1111\/j.1600-0609.2009.01247.x","article-title":"The incidence of idiopathic thrombocytopenic purpura among adults: A population-based study and literature review","volume":"83","author":"Abrahamson","year":"2009","journal-title":"Eur. J. Haematol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1111\/j.1651-2227.1997.tb08876.x","article-title":"Prevalence and treatment of chronic idiopathic thrombocytopenic purpura of childhood in Sweden","volume":"86","author":"Hedman","year":"1997","journal-title":"Acta Paediatr."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Singh, A., Uzun, G., and Bakchoul, T. (2021). Primary Immune Thrombocytopenia: Novel Insights into Pathophysiology and Disease Management. J. Clin. Med., 10.","DOI":"10.3390\/jcm10040789"},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Consolini, R., Legitimo, A., and Caparello, M.C. (2016). The Centenary of Immune Thrombocytopenia\u2014Part 1: Revising Nomenclature and Pathogenesis. Front. Pediatr., 4.","DOI":"10.3389\/fped.2016.00102"},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Gonz\u00e1lez-L\u00f3pez, T.J., Newland, A., and Provan, D. (2023). Current Concepts in the Diagnosis and Management of Adult Primary Immune Thrombocytopenia: Our Personal View. Medicina, 59.","DOI":"10.3390\/medicina59040815"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"204","DOI":"10.3390\/hematolrep16020021","article-title":"Pathophysiology, Clinical Manifestations and Diagnosis of Immune Thrombocytopenia: Contextualization from a Historical Perspective","volume":"16","author":"Bernardo","year":"2024","journal-title":"Hematol. Rep."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1111\/j.1365-2141.2010.08562.x","article-title":"ITP: A historical perspective","volume":"153","author":"Stasi","year":"2011","journal-title":"Br. J. Haematol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"894","DOI":"10.1046\/j.1365-2141.2002.03755.x","article-title":"Historical aspects and present knowledge of idiopathic thrombocytopenic purpura","volume":"119","author":"Imbach","year":"2002","journal-title":"Br. J. Haematol."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Mingot-Castellano, M.E., Canaro Hirnyk, M., S\u00e1nchez-Gonz\u00e1lez, B., \u00c1lvarez-Rom\u00e1n, M.T., B\u00e1rez-Garc\u00eda, A., Bernardo-Guti\u00e9rrez, \u00c1., Bernat-Pablo, S., Bola\u00f1os-Calder\u00f3n, E., Butta-Coll, N., and Caballero-Navarro, G. (2023). Recommendations for the Clinical Approach to Immune Thrombocytopenia: Spanish ITP Working Group (GEPTI). J. Clin. Med, 12.","DOI":"10.3390\/jcm12206422"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"3829","DOI":"10.1182\/bloodadvances.2019000966","article-title":"American Society of Hematology 2019 guidelines for immune thrombocytopenia","volume":"3","author":"Neunert","year":"2019","journal-title":"Blood Adv."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"3780","DOI":"10.1182\/bloodadvances.2019000812","article-title":"Updated international consensus report on the investigation and management of primary immune thrombocytopenia","volume":"3","author":"Provan","year":"2019","journal-title":"Blood Adv."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"457","DOI":"10.1111\/jth.12813","article-title":"Severe bleeding events in adults and children with primary immune thrombocytopenia: A systematic review","volume":"13","author":"Neunert","year":"2015","journal-title":"J. Thromb. Haemost."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1630","DOI":"10.1001\/archinte.160.11.1630","article-title":"The bleeding risk and natural history of idiopathic thrombocytopenic purpura in patients with persistent low platelet counts","volume":"160","author":"Cohen","year":"2000","journal-title":"Arch. Intern. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1111\/jth.14446","article-title":"Cardiovascular and bleeding outcomes in a population-based cohort of patients with chronic immune thrombocytopenia","volume":"17","author":"Adelborg","year":"2019","journal-title":"J. Thromb. Haemost."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1111\/jth.14227","article-title":"Risk factors for bleeding, including platelet count threshold, in newly diagnosed immune thrombocytopenia adults","volume":"16","author":"Germain","year":"2018","journal-title":"J. Thromb. Haemost."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1001\/archinte.1951.03810090041004","article-title":"Effect of corticotrophin and cortisone on the blood in various disorders in man","volume":"88","author":"Wintrobe","year":"1951","journal-title":"AMA Arch. Intern. Med."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1560","DOI":"10.1056\/NEJM199406023302203","article-title":"Response of Resistant Idiopathic Thrombocytopenic Purpura to Pulsed High-Dose Dexamethasone Therapy","volume":"330","author":"Andersen","year":"1994","journal-title":"N. Engl. J. Med."},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Zufferey, A., Kapur, R., and Semple, J. (2017). Pathogenesis and Therapeutic Mechanisms in Immune Thrombocytopenia (ITP). J. Clin. Med., 6.","DOI":"10.3390\/jcm6020016"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"e489","DOI":"10.1016\/S2352-3026(16)30109-0","article-title":"High-dose dexamethasone compared with prednisone for previously untreated primary immune thrombocytopenia: A systematic review and meta-analysis","volume":"3","author":"Mithoowani","year":"2016","journal-title":"Lancet Haematol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1007\/s12185-020-02977-9","article-title":"High-dose dexamethasone as a replacement for traditional prednisone as the first-line treatment in children with previously untreated primary immune thrombocytopenia: A prospective, randomized single-center study","volume":"112","author":"Ma","year":"2020","journal-title":"Int. J. Hematol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1056\/NEJMcp1810479","article-title":"Immune Thrombocytopenia","volume":"381","author":"Cooper","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1080\/09537104.2019.1572875","article-title":"First line treatment of adult patients with primary immune thrombocytopenia: A real-world study","volume":"31","author":"Wang","year":"2020","journal-title":"Platelets"},{"key":"ref_25","first-page":"w13593","article-title":"Treatment of immune thrombocytopenia with intravenous immunoglobulin and insights for other diseases","volume":"142","author":"Imbach","year":"2012","journal-title":"Swiss. Med. Wkly."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1228","DOI":"10.1016\/S0140-6736(81)92400-4","article-title":"High-dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura in childhood","volume":"1","author":"Imbach","year":"1981","journal-title":"Lancet"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/S0140-6736(83)91738-5","article-title":"High-dose intravenous IgG in adults with autoimmune thrombocytopenia","volume":"1","author":"Newland","year":"1983","journal-title":"Lancet"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1056\/NEJM198401263100416","article-title":"Intravenous IgG for autoimmune thrombocytopenia in pregnancy","volume":"310","author":"Newland","year":"1984","journal-title":"N. Engl. J. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/j.tmrv.2007.12.001","article-title":"The Mechanisms of Action of Intravenous Immunoglobulin and Polyclonal Anti-D Immunoglobulin in the Amelioration of Immune Thrombocytopenic Purpura: What Do We Really Know?","volume":"22","author":"Crow","year":"2008","journal-title":"Transfus. Med. Rev."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1182\/blood-2009-06-225565","article-title":"International consensus report on the investigation and management of primary immune thrombocytopenia","volume":"115","author":"Provan","year":"2010","journal-title":"Blood"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/S0140-6736(02)07275-6","article-title":"Intravenous immunoglobulin or high-dose methylprednisolone, with or without oral prednisone, for adults with untreated severe autoimmune thrombocytopenic purpura: A randomised, multicentre trial","volume":"359","author":"Godeau","year":"2002","journal-title":"Lancet"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"283","DOI":"10.3324\/haematol.10667","article-title":"The association between platelet autoantibody specificity and response to intravenous immunoglobulin G in the treatment of patients with immune thrombocytopenia","volume":"92","author":"Go","year":"2007","journal-title":"Haematologica"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1111\/jth.12524","article-title":"Association of autoantibody specificity and response to intravenous immunoglobulin G therapy in immune thrombocytopenia: A multicenter cohort study","volume":"12","author":"Peng","year":"2014","journal-title":"J. Thromb. Haemost."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"7737","DOI":"10.1038\/ncomms8737","article-title":"Desialylation is a mechanism of Fc-independent platelet clearance and a therapeutic target in immune thrombocytopenia","volume":"6","author":"Li","year":"2015","journal-title":"Nat. Commun."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1182\/bloodadvances.2019000868","article-title":"A modern reassessment of glycoprotein-specific direct platelet autoantibody testing in immune thrombocytopenia","volume":"4","author":"Rosovsky","year":"2020","journal-title":"Blood Adv."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1007\/BF00320381","article-title":"Treatment of autoimmune thrombocytopenic purpura with rhesus antibodies (anti-Rh0(D)","volume":"49","author":"Salama","year":"1984","journal-title":"Blut"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1016\/S0140-6736(83)90175-7","article-title":"Effect of intravenous immunoglobulin in immune thrombocytopenia","volume":"2","author":"Salama","year":"1983","journal-title":"Lancet"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1884","DOI":"10.1182\/blood.V77.9.1884.1884","article-title":"Intravenous anti-D treatment of immune thrombocytopenic purpura: Analysis of efficacy, toxicity, and mechanism of effect","volume":"77","author":"Bussel","year":"1991","journal-title":"Blood"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1922","DOI":"10.1182\/blood.V99.6.1922","article-title":"Does treatment with intermittent infusions of intravenous anti-D allow a proportion of adults with recently diagnosed immune thrombocytopenic purpura to avoid splenectomy?","volume":"99","author":"Cooper","year":"2002","journal-title":"Blood"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1111\/j.1365-2141.2009.08057.x","article-title":"Comparison of intravenous immune globulin and high dose anti-D immune globulin as initial therapy for childhood immune thrombocytopenic purpura","volume":"149","author":"Kane","year":"2010","journal-title":"Br. J. Haematol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1046\/j.1365-2141.2003.04567.x","article-title":"Intravenous anti-D as a treatment for immune thrombocytopenic purpura (ITP) during pregnancy","volume":"123","author":"Michel","year":"2003","journal-title":"Br. J. Haematol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"1076","DOI":"10.1046\/j.1365-2141.2001.02627.x","article-title":"A dose of 75 microg\/kg\/d of i.v. anti-D increases the platelet count more rapidly and for a longer period of time than 50 microg\/kg\/d in adults with immune thrombocytopenic purpura","volume":"112","author":"Newman","year":"2001","journal-title":"Br. J. Haematol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"2689","DOI":"10.1182\/blood.V89.8.2689","article-title":"Intravenous anti-D treatment of immune thrombocytopenic purpura: Experience in 272 patients","volume":"89","author":"Scaradavou","year":"1997","journal-title":"Blood"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1016\/S1473-0502(03)00043-0","article-title":"Mechanism of action of IVIG and anti-D in ITP","volume":"28","author":"Lazarus","year":"2003","journal-title":"Transfus. Apher. Sci."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"525","DOI":"10.3109\/09537100903144617","article-title":"Efficacy and safety of anti-D for treatment of adults with immune thrombocytopenia","volume":"20","author":"Naithani","year":"2009","journal-title":"Platelets"},{"key":"ref_46","first-page":"57","article-title":"Anti-RhD immunoglobulin in the treatment of immune thrombocytopenia","volume":"3","author":"Cheung","year":"2009","journal-title":"Biologics"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1532","DOI":"10.1182\/blood-2004-11-4303","article-title":"Disseminated intravascular coagulation associated with acute hemoglobinemia or hemoglobinuria following Rh(0)(D) immune globulin intravenous administration for immune thrombocytopenic purpura","volume":"106","author":"Gaines","year":"2005","journal-title":"Blood"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"5527","DOI":"10.1182\/blood-2006-03-004481","article-title":"A closer look at intravascular hemolysis (IVH) following intravenous anti-D for immune thrombocytopenic purpura (ITP)","volume":"109","author":"Tarantino","year":"2007","journal-title":"Blood"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1053\/j.seminhematol.2014.10.003","article-title":"Thrombopoiesis","volume":"52","author":"Kaushansky","year":"2015","journal-title":"Semin. Hematol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1117","DOI":"10.1016\/0092-8674(94)90450-2","article-title":"Identification and cloning of a megakaryocyte growth and development factor that is a ligand for the cytokine receptor Mpl","volume":"77","author":"Bartley","year":"1994","journal-title":"Cell"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"11104","DOI":"10.1073\/pnas.91.23.11104","article-title":"The purification of megapoietin: A physiological regulator of megakaryocyte growth and platelet production","volume":"91","author":"Kuter","year":"1994","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1038\/369565a0","article-title":"Cloning and expression of murine thrombopoietin cDNA and stimulation of platelet production in vivo","volume":"369","author":"Lok","year":"1994","journal-title":"Nature"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1007\/s12185-013-1382-0","article-title":"The biology of thrombopoietin and thrombopoietin receptor agonists","volume":"98","author":"Kuter","year":"2013","journal-title":"Int. J. Hematol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"4068","DOI":"10.1182\/blood.V87.10.4068.bloodjournal87104068","article-title":"Human thrombopoietin levels are high when thrombocytopenia is due to megakaryocyte deficiency and low when due to increased platelet destruction","volume":"87","author":"Emmons","year":"1996","journal-title":"Blood"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1046\/j.1365-2141.1996.d01-1702.x","article-title":"Circulating thrombopoietin level in chronic immune thrombocytopenic purpura","volume":"93","author":"Kosugi","year":"1996","journal-title":"Br. J. Haematol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1112","DOI":"10.3324\/haematol.2018.212845","article-title":"Thrombopoietin receptor agonists: Ten years later","volume":"104","author":"Ghanima","year":"2019","journal-title":"Haematologica"},{"key":"ref_57","unstructured":"(2024, February 11). Nplate Epar. Available online: https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/nplate-epar-product-information_en.pdf."},{"key":"ref_58","unstructured":"(2024, February 11). Revolade Epar. Available online: https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/revolade-epar-product-information_en.pdf."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1007\/s00277-017-2953-6","article-title":"Beyond immune thrombocytopenia: The evolving role of thrombopoietin receptor agonists","volume":"96","author":"Rodeghiero","year":"2017","journal-title":"Ann. Hematol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1016\/S0140-6736(08)60203-2","article-title":"Efficacy of romiplostim in patients with chronic immune thrombocytopenic purpura: A double-blind randomised controlled trial","volume":"371","author":"Kuter","year":"2008","journal-title":"Lancet"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1111\/bjh.12260","article-title":"Long-term treatment with romiplostim in patients with chronic immune thrombocytopenia: Safety and efficacy","volume":"161","author":"Kuter","year":"2013","journal-title":"Br. J. Haematol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1342","DOI":"10.3324\/haematol.2016.161968","article-title":"Safety and efficacy of romiplostim in splenectomized and nonsplenectomized patients with primary immune thrombocytopenia","volume":"102","author":"Cines","year":"2017","journal-title":"Haematologica"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1016\/S0140-6736(09)60402-5","article-title":"Effect of eltrombopag on platelet counts and bleeding during treatment of chronic idiopathic thrombocytopenic purpura: A randomised, double-blind, placebo-controlled trial","volume":"373","author":"Bussel","year":"2009","journal-title":"Lancet"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/S0140-6736(10)60959-2","article-title":"Eltrombopag for management of chronic immune thrombocytopenia (RAISE): A 6-month, randomised, phase 3 study","volume":"377","author":"Cheng","year":"2011","journal-title":"Lancet"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"2527","DOI":"10.1182\/blood-2017-04-748707","article-title":"Safety and efficacy of long-term treatment of chronic\/persistent ITP with eltrombopag: Final results of the EXTEND study","volume":"130","author":"Wong","year":"2017","journal-title":"Blood"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1182\/blood-2010-10-313908","article-title":"A randomized, double-blind study of romiplostim to determine its safety and efficacy in children with immune thrombocytopenia","volume":"118","author":"Bussel","year":"2011","journal-title":"Blood"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1341","DOI":"10.1007\/s00277-011-1172-9","article-title":"Romiplostim in children with chronic refractory ITP: Randomized placebo controlled study","volume":"90","author":"Elalfy","year":"2011","journal-title":"Ann. Hematol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"2283","DOI":"10.3324\/haematol.2018.202283","article-title":"Long-term treatment with romiplostim and treatment-free platelet responses in children with chronic immune thrombocytopenia","volume":"104","author":"Tarantino","year":"2019","journal-title":"Haematologica"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1016\/S0140-6736(16)00279-8","article-title":"Romiplostim in children with immune thrombocytopenia: A phase 3, randomised, double-blind, placebo-controlled study","volume":"388","author":"Tarantino","year":"2016","journal-title":"Lancet"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"e315","DOI":"10.1016\/S2352-3026(15)00114-3","article-title":"Eltrombopag for the treatment of children with persistent and chronic immune thrombocytopenia (PETIT): A randomised, multicentre, placebo-controlled study","volume":"2","author":"Bussel","year":"2015","journal-title":"Lancet Haematol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1649","DOI":"10.1016\/S0140-6736(15)61107-2","article-title":"Eltrombopag for children with chronic immune thrombocytopenia (PETIT2): A randomised, multicentre, placebo-controlled trial","volume":"386","author":"Grainger","year":"2015","journal-title":"Lancet"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1111\/ejh.12181","article-title":"Long-term safety and tolerability of romiplostim in patients with primary immune thrombocytopenia: A pooled analysis of 13 clinical trials","volume":"91","author":"Rodeghiero","year":"2013","journal-title":"Eur. J. Haematol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1111\/bjh.13266","article-title":"Use of eltrombopag after romiplostim in primary immune thrombocytopenia","volume":"169","author":"Andrade","year":"2015","journal-title":"Br. J. Haematol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"881","DOI":"10.3324\/haematol.2012.074633","article-title":"A retrospective pilot evaluation of switching thrombopoietic receptor-agonists in immune thrombocytopenia","volume":"98","author":"Khellaf","year":"2013","journal-title":"Haematologica"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1007\/s12185-014-1731-7","article-title":"Treatment patterns and clinical outcomes in patients with chronic immune thrombocytopenia (ITP) switched to eltrombopag or romiplostim","volume":"101","author":"Kuter","year":"2015","journal-title":"Int. J. Hematol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1002\/ajh.24935","article-title":"Alternate use of thrombopoietin receptor agonists in adult primary immune thrombocytopenia patients: A retrospective collaborative survey from Italian hematology centers","volume":"93","author":"Cantoni","year":"2018","journal-title":"Am. J. Hematol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"16680","DOI":"10.1038\/s41598-019-53209-y","article-title":"Deciphering predictive factors for choice of thrombopoietin receptor agonist, treatment free responses, and thrombotic events in immune thrombocytopenia","volume":"9","author":"Lozano","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_78","first-page":"E40","article-title":"Successful discontinuation of eltrombopag after complete remission in patients with primary immune thrombocytopenia","volume":"90","author":"Pascual","year":"2015","journal-title":"Am. J. Hematol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"865","DOI":"10.1111\/bjh.12888","article-title":"The temporary use of thrombopoietin-receptor agonists may induce a prolonged remission in adult chronic immune thrombocytopenia. Results of a French observational study","volume":"165","author":"Fain","year":"2014","journal-title":"Br. J. Haematol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1111\/bjh.15573","article-title":"Phase 3 randomised study of avatrombopag, a novel thrombopoietin receptor agonist for the treatment of chronic immune thrombocytopenia","volume":"183","author":"Jurczak","year":"2018","journal-title":"Br. J. Haematol."},{"key":"ref_81","unstructured":"(2024, February 11). Doptelet Epar. Available online: https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/doptelet-epar-product-information_en.pdf."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1111\/bjh.18081","article-title":"Adults with immune thrombocytopenia who switched to avatrombopag following prior treatment with eltrombopag or romiplostim: A multicentre US study","volume":"197","author":"Jiang","year":"2022","journal-title":"Br. J. Haematol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1002\/ajh.25125","article-title":"Fostamatinib for the treatment of adult persistent and chronic immune thrombocytopenia: Results of two phase 3, randomized, placebo-controlled trials","volume":"93","author":"Bussel","year":"2018","journal-title":"Am. J. Hematol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1111\/bjh.16959","article-title":"Fostamatinib is an effective second-line therapy in patients with immune thrombocytopenia","volume":"190","author":"Boccia","year":"2020","journal-title":"Br. J. Haematol."},{"key":"ref_85","unstructured":"(2024, February 11). Tavlesse Epar. Available online: https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/tavlesse-epar-product-information_en.pdf."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"2131751","DOI":"10.1080\/09537104.2022.2131751","article-title":"Recent advances in understanding spleen tyrosine kinase (SYK) in human biology and disease, with a focus on fostamatinib","volume":"34","author":"Cooper","year":"2023","journal-title":"Platelets"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"3529","DOI":"10.1182\/bloodadvances.2021006438","article-title":"Dissecting platelet proteomics to understand the pathophysiology of immune thrombocytopenia: Studies in mouse models","volume":"6","author":"Meinders","year":"2022","journal-title":"Blood Adv."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"20406207221147777","DOI":"10.1177\/20406207221147777","article-title":"Use and positioning of fostamatinib in the management of primary chronic immune thrombocytopenia: An Italian expert opinion","volume":"14","author":"Lucchesi","year":"2023","journal-title":"Ther. Adv. Hematol."},{"key":"ref_89","unstructured":"(2024, February 12). Mabthera Epar. Available online: https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/mabthera-epar-product-information_en.pdf."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"1124","DOI":"10.3324\/haematol.2019.218883","article-title":"Rituximab in the treatment of immune thrombocytopenia: What is the role of this agent in 2019?","volume":"104","author":"Lucchini","year":"2019","journal-title":"Haematologica"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"584","DOI":"10.1182\/blood.V69.2.584.584","article-title":"Monoclonal antibody 1F5 (anti-CD20) serotherapy of human B cell lymphomas","volume":"69","author":"Press","year":"1987","journal-title":"Blood"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"3490","DOI":"10.1182\/blood.V92.9.3490","article-title":"Rituxan in the treatment of cold agglutinin disease","volume":"92","author":"Lee","year":"1998","journal-title":"Blood"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1002\/ajh.1112","article-title":"Successful treatment with Rituximab for relapsing immune thrombocytopenic purpura (ITP) associated with low-grade non-Hodgkin\u2019s lymphoma","volume":"67","author":"Shvidel","year":"2001","journal-title":"Am. J. Hematol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1182\/blood.V98.4.952","article-title":"Rituximab chimeric anti-CD20 monoclonal antibody treatment for adults with chronic idiopathic thrombocytopenic purpura","volume":"98","author":"Stasi","year":"2001","journal-title":"Blood"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.jpeds.2004.09.004","article-title":"Chronic immune thrombocytopenic purpura in children: Assessment of rituximab treatment","volume":"146","author":"Wang","year":"2005","journal-title":"J. Pediatr."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"5989","DOI":"10.1182\/blood-2011-11-393975","article-title":"Outcomes 5 years after response to rituximab therapy in children and adults with immune thrombocytopenia","volume":"119","author":"Patel","year":"2012","journal-title":"Blood"},{"key":"ref_97","doi-asserted-by":"crossref","unstructured":"Dong, Y., Yue, M., and Hu, M. (2021). The Efficacy and Safety of Different Dosages of Rituximab for Adults with Immune Thrombocytopenia: A Systematic Review and Meta-Analysis. BioMed Res. Int., 2021.","DOI":"10.1155\/2021\/9992086"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"690","DOI":"10.1080\/09537104.2019.1624706","article-title":"The efficacy and safety of low-dose rituximab in immune thrombocytopenia: A systematic review and meta-analysis","volume":"30","author":"Li","year":"2019","journal-title":"Platelets"},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"3228","DOI":"10.1182\/blood-2014-06-582346","article-title":"Safety and efficacy of rituximab in adult immune thrombocytopenia: Results from a prospective registry including 248 patients","volume":"124","author":"Khellaf","year":"2014","journal-title":"Blood"},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1002\/ajh.23518","article-title":"Efficacy and safety of rituximab given at 1,000 mg on days 1 and 15 compared to the standard regimen to treat adult immune thrombocytopenia","volume":"88","author":"Ebbo","year":"2013","journal-title":"Am. J. Hematol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"2755","DOI":"10.1182\/blood-2009-07-229815","article-title":"Dexamethasone plus rituximab yields higher sustained response rates than dexamethasone monotherapy in adults with primary immune thrombocytopenia","volume":"115","author":"Zaja","year":"2010","journal-title":"Blood"},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"1976","DOI":"10.1182\/blood-2012-09-455691","article-title":"Rituximab and dexamethasone vs. dexamethasone monotherapy in newly diagnosed patients with primary immune thrombocytopenia","volume":"121","author":"Gudbrandsdottir","year":"2013","journal-title":"Blood"},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"1264","DOI":"10.3324\/haematol.2013.103291","article-title":"Rituximab and three dexamethasone cycles provide responses similar to splenectomy in women and those with immune thrombocytopenia of less than two years duration","volume":"99","author":"Bussel","year":"2014","journal-title":"Haematologica"},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"e75","DOI":"10.1016\/S2352-3026(15)00003-4","article-title":"Rituximab plus standard of care for treatment of primary immune thrombocytopenia: A systematic review and meta-analysis","volume":"2","author":"Chugh","year":"2015","journal-title":"Lancet Haematol."},{"key":"ref_105","first-page":"1451","article-title":"Verschwinden der hamorrhagischen diathese bei einem falle von essentieller thrombopenie (frank) nach milzexstiparation: Splenogene thrombolytische purpura","volume":"29","author":"Kaznelson","year":"1916","journal-title":"Wien. Klin. Wochenschr."},{"key":"ref_106","first-page":"1","article-title":"Demonstration of a thrombocytopenic factor in the blood of patients with thrombocytopenic purpura","volume":"38","author":"Harrington","year":"1951","journal-title":"J. Lab. Clin. Med."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"E267","DOI":"10.1002\/ajh.24310","article-title":"Have splenectomy rate and main outcomes of ITP changed after the introduction of new treatments? A monocentric study in the outpatient setting during 35 years","volume":"91","author":"Palandri","year":"2016","journal-title":"Am. J. Hematol."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"1172","DOI":"10.1182\/blood-2017-09-742353","article-title":"Splenectomy for immune thrombocytopenia: Down but not out","volume":"131","author":"Chaturvedi","year":"2018","journal-title":"Blood"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"2623","DOI":"10.1182\/blood-2004-03-1168","article-title":"Splenectomy for adult patients with idiopathic thrombocytopenic purpura: A systematic review to assess long-term platelet count responses, prediction of response, and surgical complications","volume":"104","author":"Kojouri","year":"2004","journal-title":"Blood"},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"4782","DOI":"10.1182\/blood-2012-12-467068","article-title":"Splenectomy and the incidence of venous thromboembolism and sepsis in patients with immune thrombocytopenia","volume":"121","author":"Boyle","year":"2013","journal-title":"Blood"},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1111\/ejh.12146","article-title":"Safety and efficacy of splenectomy in over 65-yrs-old patients with immune thrombocytopenia","volume":"91","author":"Escalante","year":"2013","journal-title":"Eur. J. Haematol."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1002\/ajh.26378","article-title":"Splenectomy for primary immune thrombocytopenia revisited in the era of thrombopoietin receptor agonists: New insights for an old treatment","volume":"97","author":"Mageau","year":"2022","journal-title":"Am. J. Hematol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1002\/ajh.24702","article-title":"Newly diagnosed immune thrombocytopenia adults: Clinical epidemiology, exposure to treatments, and evolution. Results of the CARMEN multicenter prospective cohort","volume":"92","author":"Moulis","year":"2017","journal-title":"Am. J. Hematol."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1002\/pbc.20453","article-title":"Childhood ITP: 12 months follow-up data from the prospective registry I of the Intercontinental Childhood ITP Study Group (ICIS)","volume":"46","author":"Imbach","year":"2006","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1182\/blood.2019003599","article-title":"Identifying and treating refractory ITP: Difficulty in diagnosis and role of combination treatment","volume":"135","author":"Miltiadous","year":"2020","journal-title":"Blood"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1007\/s00277-022-04786-y","article-title":"Refractory primary immune thrombocytopenia (ITP): Current clinical challenges and therapeutic perspectives","volume":"101","author":"Vianelli","year":"2022","journal-title":"Ann. Hematol."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1111\/bjh.19075","article-title":"Refractory immune thrombocytopenia in adults: Towards a new definition","volume":"203","author":"Arnold","year":"2023","journal-title":"Br. J. Haematol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"1625","DOI":"10.1182\/blood-2016-03-704734","article-title":"Characteristics, outcome, and response to therapy of multirefractory chronic immune thrombocytopenia","volume":"128","author":"Moulis","year":"2016","journal-title":"Blood"},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"e37","DOI":"10.1111\/bjh.16426","article-title":"Combination of thrombopoietin receptor agonists, immunosuppressants and intravenous immunoglobulin as treatment of severe refractory immune thrombocytopenia in adults and children","volume":"189","author":"Gudbrandsdottir","year":"2020","journal-title":"Br. J. Haematol."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1111\/bjh.18893","article-title":"Combining thrombopoietin receptor agonists with immunosuppressive drugs in adult patients with multirefractory immune thrombocytopenia, an update on the French experience","volume":"202","author":"Crickx","year":"2023","journal-title":"Br. J. Haematol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1111\/bjh.19078","article-title":"Novel therapeutics and future directions for refractory immune thrombocytopenia","volume":"203","author":"Neufeld","year":"2023","journal-title":"Br. J. Haematol."},{"key":"ref_122","doi-asserted-by":"crossref","unstructured":"Audia, S., and Bonnotte, B. (2021). Emerging Therapies in Immune Thrombocytopenia. J. Clin. Med., 10.","DOI":"10.3390\/jcm10051004"},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1111\/bjh.17872","article-title":"Novel therapies for immune thrombocytopenia","volume":"196","author":"Kuter","year":"2022","journal-title":"Br. J. Haematol."},{"key":"ref_124","doi-asserted-by":"crossref","unstructured":"Provan, D., and Semple, J.W. (2022). Recent advances in the mechanisms and treatment of immune thrombocytopenia. EBioMedicine, 76.","DOI":"10.1016\/j.ebiom.2022.103820"},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"4136","DOI":"10.1182\/bloodadvances.2020002003","article-title":"Phase 2 multiple-dose study of an FcRn inhibitor, rozanolixizumab, in patients with primary immune thrombocytopenia","volume":"4","author":"Robak","year":"2020","journal-title":"Blood Adv."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"1648","DOI":"10.1016\/S0140-6736(23)01460-5","article-title":"Efficacy and safety of the neonatal Fc receptor inhibitor efgartigimod in adults with primary immune thrombocytopenia (ADVANCE IV): A multicentre, randomised, placebo-controlled, phase 3 trial","volume":"402","author":"Broome","year":"2023","journal-title":"Lancet"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1056\/NEJMoa2110297","article-title":"Rilzabrutinib, an Oral BTK Inhibitor, in Immune Thrombocytopenia","volume":"386","author":"Kuter","year":"2022","journal-title":"N. Engl. J. Med."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"987","DOI":"10.1182\/bloodadvances.2021006864","article-title":"Safety and efficacy of classical complement pathway inhibition with sutimlimab in chronic immune thrombocytopenia","volume":"7","author":"Broome","year":"2023","journal-title":"Blood Adv."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"1141","DOI":"10.1097\/QAD.0000000000001036","article-title":"Thrombopoietin receptor agonists in conjunction with oseltamivir for immune thrombocytopenia","volume":"30","author":"Bernardino","year":"2016","journal-title":"AIDS"},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"495","DOI":"10.3109\/09537104.2014.948838","article-title":"Successful treatment with oseltamivir phosphate in a patient with chronic immune thrombocytopenia positive for anti-GPIb\/IX autoantibody","volume":"26","author":"Shao","year":"2015","journal-title":"Platelets"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"e289","DOI":"10.1016\/S2352-3026(21)00030-2","article-title":"Dexamethasone plus oseltamivir versus dexamethasone in treatment-naive primary immune thrombocytopenia: A multicentre, randomised, open-label, phase 2 trial","volume":"8","author":"Sun","year":"2021","journal-title":"Lancet Haematol."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1160\/TH10-11-0744","article-title":"The effect of decitabine on megakaryocyte maturation and platelet release","volume":"106","author":"Wang","year":"2011","journal-title":"Thromb. Haemost."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1160\/TH14-04-0342","article-title":"Low-dose decitabine promotes megakaryocyte maturation and platelet production in healthy controls and immune thrombocytopenia","volume":"113","author":"Zhou","year":"2015","journal-title":"Thromb. Haemost."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"1374","DOI":"10.1002\/ajh.25646","article-title":"A prospective, multicenter study of low dose decitabine in adult patients with refractory immune thrombocytopenia","volume":"94","author":"Zhou","year":"2019","journal-title":"Am. J. 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