Clinical and epidemiological investigation of the treatment of patients with inhibitory alloatibodies in hemophilia type A treated at a blood center in northeastern Brazil
DOI:
https://doi.org/10.33448/rsd-v11i4.27435Keywords:
Hemophilia A; Hemotherapy service; Blood banks.Abstract
The present study aims to analyze the epidemiological profile, diagnosis and treatment of hemophiliac patients who developed inhibitors and who are treated by the Hemotherapy Center of Sergipe (HEMOSE). The research consists of a retrospective analysis based on information contained in the medical records of patients with hemophilia A who were treated in the outpatient sector of HEMOSE during the period from January 2010 to January 2018. For this purpose, variables such as gender were evaluated , age, location, color, severity, symptoms, inhibitor titer, and treatment. Of the patients evaluated, 17 (18%) had inhibitors, and, of this total, 11 (64.7%) were brown. In addition, most patients were diagnosed with hemophilia A and detected the presence of inhibitor in the age group between 0 and 10 years. Regarding the severity of the disease, 11 (64.7%) had the severe form. With regard to treatment before the development of inhibitors, the majority, 10 (59%), used an association of Factor VIII with Factor VIIIr. It is also noteworthy that the majority of patients developed low titer antibodies, with a rate of 82% (14). It is also considered that the results found in this study are in accordance with what is found in the literature on the subject and that the information obtained provides a better understanding of the profile of patients with hemophilia A who have inhibitors, which can then improve care. of these patients.
References
Berntorp, E. Negrier, C. Gozzi, P. Blaas, M., & Lethagen S. (2016). Dosing regimens, FVIII levels and estimated haemostatic protection with special focus on rFVIIIFc. Haemophilia. 22(2), 389-396.
Brasil. Ministério da Saúde. (2009). Secretaria de Atenção à Saúde. Hemofilia Congênita e Inibidor: Manual de Diagnóstico e Tratamento de Eventos Hemorrágicos. Departamento de Atenção Especializada. 1ª Ed. Brasília, DF.
Brasil. Ministério da Saúde. (2015). Brasil atinge meta da OMS e amplia assistência aos hemofílicos. 1-10.
Brasil. Ministério da Saúde. (2015). Secretaria de Atenção à Saúde. Departamento de Atenção Especializada. Manual de Hemofilia. 2ª Ed. Brasília, DF.
Brasil. Ministério da Saúde. (2017). Departamento de Atenção Especializada e Temática. Perfil das Coagulopatias Hereditárias no Brasil. Secretaria de Atenção à Saúde. Brasília, DF. 1ª Ed.
Cabral, D.R. (2015). Hemofilia: aspectos fisiológicos, moleculares, genéticos e possíveis tratamentos. Universidade Federal do Paraná, Curitiba. 28.
Carcao, M., et al. (2018). Recombinant factor VIII Fc fusion protein for immune tolerance induction in patients with severe haemophilia A with inhibitors—A retrospective analysis. Haemophilia. (24), 1-8.
Carvalho, R.A., Silva, P.H., & Henneberg, R. (2016). Incidence of factor VIII inhibitory antibodies in patients with hemophilia A seen at HEMOCE, Ceará, Brazil. Bras Patol Med Lab. 52(6), 382-386.
Duncan, E., Collecutt, M., & Street, A. (2013). Nijmegen-Bethesda assay to measure factor VIII inhibitors. Methods Mol Biol. 992, 321-33.
Ettinger, R.A., et al. (2018). FVIII proteins with a modified immunodominant T-cell epitope exhibit reduced immunogenicity and normal FVIII activity. Blood Advances, Washington DC. 2(4), 309-322.
Federação Brasileira De Hemofilia – FBH. (2017). Hemofilia Denota Cuidados Integrais. Caxias do Sul, RS. 2(2), 8-12.
Ferreira, L., et al. (2006). Genomic ancestry of a sample population from the State of Sao Paulo, Brazil. American Journal of Human Biology. 18(5), 702:705.
Goudemand, J., et al. (2006). Influence of the type of factor VIII concentrate on the incidence of factor VIII inhibitors in previously untreated patients with severe hemophilia A. Blood. 107, 46-51.
Gouw, S.C., & Fijnvandraat, K. (2013). Identifying nongenetic risk factors for inhibitor development in severe hemophilia A. Semin Thromb Hemost. 39, 740–751.
Gouw, S.C., et al. (2007). Recombinant versus plasma-derived factor VIII products and the development of inhibitors in previously untreated patients with severe hemophilia A: The canal cohort study. Blood. 108(11), 4693-4697.
Guedes, V.G. (2016). Avaliação da adesão ao tratamento profilático na hemofilia: Estudo transversal no interior do estado de São Paulo (Brasil). Universidade Estadual Paulista – Botucatu. 121-132).
Key, N.S. (2004). Inhibitors in congenital coagulation disorders. Br J Haematol. 127, 379-91.
Morado, M., Villar, A., Jimenez, Y.V., Quintana, M., & Hernandez, N.F. (2005). Prophylatic treatment effects on inhibitor risk: experience in one centre. Haemophilia. 11, 79-83.
Muniz, N., et al. (2008). Genomic ancestry in urban afro-brazilians. Annals of Human Biology. 35(1), 104-111.
Peyvandi, F., et al. (2016). A Randomized Trial of Factor VIII and Neutralizing Antibodies in Hemophilia A. N Engl J Med. 374(21), 2054-64.
Rodrigues, S.H.L. (2015). Perfil de utilização de medicamentos pró-coagulantes bypassing disponibilizados no SUS para tratamento das coagulopatias. Universidade Federal da Bahia, Salvador. 2015. 46.
Ryu, J.E.U.N., et al. (2015). Immune tolerance induction in patients with severe hemophilia A with inhibitors. Blood Research. 50(4), 309-322.
Santos, A. (2010). Caracterização de aspectos genéticos e imunológicos envolvidos no desenvolvimento de inibidores em Hemofilia A e B. Universidade Estadual de Campinas, Campinas. 185-192.
Sousa, C.I.F. (2010). Tratamento e profilaxia da hemofilia na criança. Universidade do Porto, Porto. 29.
Souza, T.B., et al. (2011). Farmacogenética do desenvolvimento de anticorpos inibidores do fator VIII na hemofilia A. Revista Científica da FMC. 6(1), 9-13.
Wight, J., & Paisley, S. (2003). The epidemiology of inhibitors in haemophilia A: a systematic review. Haemophilia. 9, 418–35.
Witmer, C., & Young, G. (2013). Factor VIII inhibitors in hemophilia A: rationale and latest evidence. Ther Adv Hematol. 4(1), 59-72.
World Federation Of Hemophilia – WFH. (2017). Report on the Annual Global Survey 2016. World Federation of Hemophilia. 1(8), 74.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2022 Mikaely Rodrigues dos Santos; Alessandra Faustino da Conceição Bezerra; Weber de Santana Teles; Max Cruz da Silva; Ruth Cristini Torres; Silvia Maria da Silva Sant'ana Rodrigues; Ângela Maria Melo Sá Barros; Paulo Celso Curvelo Santos Junior; Taíssa Alice Soledade Calasans; Aline Barreto Hora
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
1) Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
2) Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
3) Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.