Treatment of primary immune thrombocytopenia in adults: a literature review
DOI:
https://doi.org/10.47456/rbps.v26isupl_1.44404Keywords:
Immune thrombocytopenia, Corticosteroids, Splenectomy, RituximabAbstract
Introduction: Primary Immune Thrombocytopenia is an autoimmune disorder characterized by isolated thrombocytopenia and consequent hemorrhagic phenomen, whitch mechanism is related to platelet destruction by autoantibodies, as well as dysregulation of magakariopoiesis. The clinical presentation is characterized by thrombocytopenia <100,000/mm³ and the presence of varied mucocutaneous bleeding, requiring the exclusion of other causes of thrombocytopenia for diagnosis. Treatment aims to reduce bleeding risk by increasing platelet levels, through therapies that involve corticosteroids, immunosuppressants, thrombopoietin receptor agonist (ARTPO) and splenectomy. Objectives: Review and describe treatment in the setting of both newly diagnosed disease and persistent/chronic disease, bringing together international protocols and those published by the Brazilian Ministry of Health. Methods: Bibliographic review carried out in the Pubmed database, considering articles with the keyword “Immune Thrombocytopenia treatment in adults”. Conclusion: The management of primary immune thrombocytopenia in adults can be challenging, given the high rate of progression to the chronic form, requiring recurrent treatments in relapse scenarios and therapies with some degree of immunosuppression, impacting the patient’s morbidity and quality of life.
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References
Cooper N, Ghanima W. Immune thrombocytopenia. N Engl J Med. 2019;381(10):945–55.
Chang M, Nakagawa PA, Williams SA, et al. Immune thrombo¬cytopenic purpura (ITP) plasma and purified ITP monoclonal autoantibodies inhibit megakaryocytopoiesis in vitro. Blood. 2003;102(3):887–95.
Liu X, Hou Y, Peng J. Advances in immunopathogenesis of adult immune thrombocytopenia. Front Med. 2013;7(4):418–24.
McCrae K. Immune thrombocytopenia: no longer ‘idiopathic’. Cleve Clin J Med. 2011;78(6):358–73.
McKenzie CG, Guo L, Freedman J, Semple JW. Cellular immune dysfunction in immune thrombocytopenia (ITP). Br J Haematol. 2013;163(1):10–23.
Rodeghiero F, Stasi R, Gernsheimer T, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009;113:2386.
Neunert C, Noroozi N, Norman G, et al. Severe bleeding events in adults and children with primary immune thrombocytope¬nia: a systematic review. J Thromb Haemost. 2015;13:457.
Cortelazzo S, Finazzi G, Buelli M, et al. High risk of severe bleed¬ing in aged patients with chronic idiopathic thrombocytopenic purpura. Blood. 1991;77:31.
Ministério da Saúde. Protocolo clínico e diretrizes terapêuticas de púrpura trombocitopênica idiopática. Brasília: Ministério da Saúde; 2023.
Neunert C, Terrell DR, Arnold DM, et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019;3:3829.
Bussel JB, Cooper N, Boccia R, Zaja F, Newland A. Immune thrombocytopenia. Blood. 2020;135(9):605–17.
Arnold DM, Cuker A. Initial treatment of immune thrombocy¬topenia (ITP) in adults. UpToDate. 2022.
Mithoowani S, Cervi A, Shah N, et al. Management of major bleeds in patients with immune thrombocytopenia. J Thromb Haemost. 2020;18:1783.
Carcao M, Silva M, David M, et al. A randomized, dou¬ble-blinded, placebo-controlled study of IVIG vs. IVIG with high dose methylprednisolone in rapidly augmenting platelet counts in childhood ITP. Blood. 2016;128(22).
Godeau B, Chevret S, Varet B, et al. Intravenous immunoglobu¬lin or high-dose methylprednisolone, with or without oral pred¬nisone, for adults with untreated severe autoimmune throm¬bocytopenic purpura: a randomised, multicentre trial. Lancet. 2002;359(9300):23–9.
Goel R, Ness PM, Takemoto CM, et al. Platelet transfusions in platelet consumptive disorders are associated with arterial thrombosis and in-hospital mortality. Blood. 2015;125:1470.
Cines DB, Bussel JB. How I treat idiopathic thrombocytopenic purpura (ITP). Blood. 2005;106(7):2244–51.
Wei Y, Ji XB, Wang YW, et al. High-dose dexamethasone vs pred¬nisone for treatment of adult immune thrombocytopenia: a pro¬spective multicenter randomized trial. Blood. 2016;127(3):296– 302.
Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115(2):168–86.
Arnold DM, Cuker A. Second-line and subsequent therapies for immune thrombocytopenia (ITP) in adults. UpToDate. 2023.
Bylsma LC, Fryzek JP, Cetin K, et al. Systematic literature review of treatments used for adult immune thrombocytopenia in the second-line setting. Am J Hematol. 2019;94:118.
George JN, Terrell DR. New thrombopoietic agents: a new era in the management of patients with thrombocytopenia. Hemato¬logica. 2008;93:1445.
Zeng Y, Duan X, Xu J, Ni X. TPO receptor agonist for chronic immune thrombocytopenic purpura. Cochrane Database Syst Rev. 2011;CD008235.
Cheng G, Saleh MN, Marcher C, et al. Eltrombopag for treat¬ment of immune thrombocytopenia (RAISE): a phase 3, ran¬domized, 6-month study. Lancet. 2011;377(9763):393–402.
Tremblay G, Dolph M, Roy AN, et al. Cost-effectiveness of eltrombopag for the treatment of immune thrombocytopenia in the United States. Clin Ther. 2020;42(5):860–72.e8.
Kuter DJ, Bussel JB, Newland A, et al. Long-term treatment with romiplostim in patients with immune thrombocytopenia: safety and efficacy. Br J Haematol. 2013;161:411.
Bussel JB, Kuter DJ, George JN, et al. AMG 531, a thrombo¬poiesis-stimulating protein, for chronic ITP. N Engl J Med. 2006;355:1672.
Shirasugi Y, Ando K, Miyazaki K, et al. Romiplostim for the treatment of chronic immune thrombocytopenia in adult Japa¬nese patients: a double-blind, randomized Phase III clinical trial. Int J Hematol. 2011;94:71.
George JN, Mathias SD, Go RS, et al. Improved quality of life for patients treated with romiplostim with chronic immune throm¬bocytopenic purpura: results from two randomized controlled trials. Br J Haematol. 2009;144:409.
Kuter DJ, Allen LF. Avatrombopag, an oral thrombopoie¬tin receptor agonist: results from two Phase 1, double-blind, dose-escalation, placebo-controlled studies. Br J Haematol. 2018;183:466.
Arnold DM, Dentali F, Crowther MA, et al. Systematic review: efficacy and safety of rituximab in adults with idiopathic throm¬bocytopenic purpura. Ann Intern Med. 2007;146(1):25–33.
Patel VL, Mahévas M, Lee SY, et al. Outcomes 5 years after response to rituximab therapy in children and adults with immune thrombocytopenia. Blood. 2012;119(25):5989–95.
Medeot M, Zaja F, Vianelli N, et al. Rituximab therapy in adults with relapsed or refractory immune thrombocytopenic purpura: long-term follow-up results. Eur J Haematol. 2008;81(3):165–69.
Vianelli N, Palandri F, Polverelli N, et al. Splenectomy as a cura¬tive treatment for immune thrombocytopenia: a retrospective analysis of 233 patients with a minimum follow-up of 10 years. Haematologica. 2013;98(6):875–80.
Gonzalez-Porras JR, Escalante F, Pardal E, et al. Safety and effi¬cacy of splenectomy in over 65-yrs-old patients with immune thrombocytopenia. Eur J Haematol. 2013;91:236.
Bussel J, Arnold DM, Duliege AM, et al. Long-term fostamatinib treatment of adults with immune thrombocytopenia during the phase 3 clinical trial program. Am J Hematol. 2019;94(5):546–53.
Fundação Pró-Sangue. Manual de transfusão. São Paulo: Fundação Pró-Sangue; 2018.
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