Maior reposição de Fator VIII (FVIII) da coagulação em pacientes com Hemofilia A moderada dos grupos sanguíneos não-O

Autores

DOI:

https://doi.org/10.33448/rsd-v13i11.47484

Palavras-chave:

Hemofilia A; Grupo Sanguíneo ABO; Fator de Von Willebrand; Cofator de Ristocetina; Tratamento de Reposição de FVIII.

Resumo

Objetivo: O presente estudo investigou a associação entre o grupo sanguíneo ABO e o consumo anual de concentrado de fator VIII (FVIII) da coagulação (UI/kg/ano) em pacientes com hemofilia A leve e moderada, atendidos em dois centros de hemofilia de referência no Brasil (Hemocentro do Pará e Hemocentro da Unicamp). Métodos: Níveis plasmáticos do antígeno do fator de von Willebrand (FVW:Ag), cofator de ristocetina (FVW:RCo) e FVIII coagulante (FVIII:C) foram realizados para investigar a correlação entre essas variáveis e os imunofenótipos ABO. Resultados e discussão: O consumo de FVIII (UI/kg/ano) comparado entre pacientes com hemofilia A moderada com grupo sanguíneo O e não-O demonstrou maior consumo de FVIII entre os pacientes com grupo sanguíneo não-O (p=0,018). Na análise desse grupo, os níveis plasmáticos de FVW:C foram estatisticamente maiores (p=0,045) em comparação aos pacientes com hemofilia A moderada com grupo sanguíneo O. Conclusão: Com base em nossos achados, o grupo sanguíneo ABO não é apenas um possível indicador para a farmacocinética do FVIII, mas também pode influenciar o fenótipo hemorrágico moderado da hemofilia A.

Referências

Albánez, S., Ogiwara, K., Michels, A., Hopman, W., Grabell, J., James, P., & Lillicrap, D. (2016). Aging and ABO blood type influence von Willebrand factor and factor VIII levels through interrelated mechanisms. Journal of thrombosis and haemostasis : JTH, 14(5), 953–963. https://doi.org/10.1111/jth.13294

Ayres, M., Ayres-Junior, F. L., & Ayres, D. L. (2007). BioEstat 5.0: Aplicações estatísticas nas áreas das ciências biológicas e médicas (5ª ed.). Belém: Sociedade Civil Mamirauá.

Bowen D. J. (2003). An influence of ABO blood group on the rate of proteolysis of von Willebrand factor by ADAMTS13. Journal of thrombosis and haemostasis : JTH, 1(1), 33–40. https://doi.org/10.1046/j.1538-7836.2003.00007.x

Carcao, M. D., van den Berg, H. M., Ljung, R., Mancuso, M. E., & PedNet and the Rodin Study Group (2013). Correlation between phenotype and genotype in a large unselected cohort of children with severe hemophilia A. Blood, 121(19), 3946–S1. https://doi.org/10.1182/blood-2012-11-469403

d'Oiron, R., Pipe, S. W., & Jacquemin, M. (2008). Mild/moderate haemophilia A: new insights into molecular mechanisms and inhibitor development. Haemophilia : the official journal of the World Federation of Hemophilia, 14 Suppl 3, 138–146. https://doi.org/10.1111/j.1365-2516.2008.01730.x

Federici, A. B., Elder, J. H., De Marco, L., Ruggeri, Z. M., & Zimmerman, T. S. (1984). Carbohydrate moiety of von Willebrand factor is not necessary for maintaining multimeric structure and ristocetin cofactor activity but protects from proteolytic degradation. The Journal of clinical investigation, 74(6), 2049–2055. https://doi.org/10.1172/JCI111628

Fijnvandraat, K., Peters, M., & ten Cate, J. W. (1995). Inter-individual variation in half-life of infused recombinant factor VIII is related to pre-infusion von Willebrand factor antigen levels. British journal of haematology, 91(2), 474–476. https://doi.org/10.1111/j.1365-2141.1995.tb05325.x

Fischer, K., Pendu, R., van Schooten, C. J., van Dijk, K., Denis, C. V., van den Berg, H. M., & Lenting, P. J. (2009). Models for prediction of factor VIII half-life in severe haemophiliacs: distinct approaches for blood group O and non-O patients. PloS one, 4(8), e6745. https://doi.org/10.1371/journal.pone.0006745

Franchini, M., Mengoli, C., Marano, G., Pupella, S., Mannucci, P. M., & Liumbruno, G. M. (2018). The importance of ABO blood group in pharmacokinetic studies in haemophilia A. Haemophilia : the official journal of the World Federation of Hemophilia, 24(3), e122–e123. https://doi.org/10.1111/hae.13437

Gallinaro, L., Cattini, M. G., Sztukowska, M., Padrini, R., Sartorello, F., Pontara, E., Bertomoro, A., Daidone, V., Pagnan, A., & Casonato, A. (2008). A shorter von Willebrand factor survival in O blood group subjects explains how ABO determinants influence plasma von Willebrand factor. Blood, 111(7), 3540–3545. https://doi.org/10.1182/blood-2007-11-122945

Garcia-Martínez, I., Borràs, N., Martorell, M., Parra, R., Altisent, C., Ramírez, L., Álvarez-Román, M. T., Nuñez, R., Megias-Vericat, J. E., Corrales, I., Alonso, S., & Vidal, F. (2020). Common Genetic Variants in ABO and CLEC4M Modulate the Pharmacokinetics of Recombinant FVIII in Severe Hemophilia A Patients. Thrombosis and haemostasis, 120(10), 1395–1406. https://doi.org/10.1055/s-0040-1714214

Gill, J. C., Endres-Brooks, J., Bauer, P. J., Marks, W. J., Jr, & Montgomery, R. R. (1987). The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood, 69(6), 1691–1695.

Greenwell P. (1997). Blood group antigens: molecules seeking a function?. Glycoconjugate journal, 14(2), 159–173. https://doi.org/10.1023/a:1018581503164

Jacquemin, M., De Maeyer, M., D'Oiron, R., Lavend'Homme, R., Peerlinck, K., & Saint-Remy, J. M. (2003). Molecular mechanisms of mild and moderate hemophilia A. Journal of thrombosis and haemostasis : JTH, 1(3), 456–463. https://doi.org/10.1046/j.1538-7836.2003.00088.x

Kano, T., Kondo, K., Hamako, J., Matsushita, F., Sakai, K., & Matsui, T. (2018). Effects of plasma glycosyltransferase on the ABO(H) blood group antigens of human von Willebrand factor. International journal of hematology, 108(2), 139–144. https://doi.org/10.1007/s12185-018-2452-0

Kaufman R. J. (1998). Post-translational modifications required for coagulation factor secretion and function. Thrombosis and haemostasis, 79(6), 1068–1079.

Lenting, P. J., VAN Schooten, C. J., & Denis, C. V. (2007). Clearance mechanisms of von Willebrand factor and factor VIII. Journal of thrombosis and haemostasis : JTH, 5(7), 1353–1360. https://doi.org/10.1111/j.1538-7836.2007.02572.x

Lenting, P. J., van Mourik, J. A., & Mertens, K. (1998). The life cycle of coagulation factor VIII in view of its structure and function. Blood, 92(11), 3983–3996.

Lillicrap D. (2008). Extending half-life in coagulation factors: where do we stand?. Thrombosis research, 122 Suppl 4, S2–S8. https://doi.org/10.1016/S0049-3848(08)70027-6

Liumbruno, G. M., & Franchini, M. (2013). Beyond immunohaematology: the role of the ABO blood group in human diseases. Blood transfusion = Trasfusione del sangue, 11(4), 491–499. https://doi.org/10.2450/2013.0152-13

Lunghi, B., Morfini, M., Martinelli, N., Linari, S., Castaman, G., & Bernardi, F. (2022). Combination of CLEC4M rs868875 G-Carriership and ABO O Genotypes May Predict Faster Decay of FVIII Infused in Hemophilia A Patients. Journal of clinical medicine, 11(3), 733. https://doi.org/10.3390/jcm11030733

Miller, C. H., Haff, E., Platt, S. J., Rawlins, P., Drews, C. D., Dilley, A. B., & Evatt, B. (2003). Measurement of von Willebrand factor activity: relative effects of ABO blood type and race. Journal of thrombosis and haemostasis : JTH, 1(10), 2191–2197. https://doi.org/10.1046/j.1538-7836.2003.00367.x

O'Donnell, J., & Laffan, M. A. (2001). The relationship between ABO histo-blood group, factor VIII and von Willebrand factor. Transfusion medicine (Oxford, England), 11(4), 343–351. https://doi.org/10.1046/j.1365-3148.2001.00315.x

Orstavik, K. H., Magnus, P., Reisner, H., Berg, K., Graham, J. B., & Nance, W. (1985). Factor VIII and factor IX in a twin population. Evidence for a major effect of ABO locus on factor VIII level. American journal of human genetics, 37(1), 89–101.

Preston, A. E., & Barr, A. (1964). The plasma concentration of factor VIII in the normal population. II. The effects of age, sex and blood group. British Journal of Haematology, 10, 238–245. https://doi.org/10.1111/j.1365-2141.1964.tb00698.x

Sodetz, J. M., Paulson, J. C., & McKee, P. A. (1979). Carbohydrate composition and identification of blood group A, B, and H oligosaccharide structures on human Factor VIII/von Willebrand factor. The Journal of biological chemistry, 254(21), 10754–10760.

Souto, J. C., Almasy, L., Muñiz-Diaz, E., Soria, J. M., Borrell, M., Bayén, L., Mateo, J., Madoz, P., Stone, W., Blangero, J., & Fontcuberta, J. (2000). Functional effects of the ABO locus polymorphism on plasma levels of von Willebrand factor, factor VIII, and activated partial thromboplastin time. Arteriosclerosis, thrombosis, and vascular biology, 20(8), 2024–2028. https://doi.org/10.1161/01.atv.20.8.2024

Swystun, L. L., Ogiwara, K., Rawley, O., Brown, C., Georgescu, I., Hopman, W., Labarque, V., Male, C., Thom, K., Blanchette, V. S., Carcao, M. D., & Lillicrap, D. (2019). Genetic determinants of VWF clearance and FVIII binding modify FVIII pharmacokinetics in pediatric hemophilia A patients. Blood, 134(11), 880–891. https://doi.org/10.1182/blood.2019000190

Toassi, R. S. C. & Petry, P. C. (2021). Metodologia científica aplicada a área de saúde. Ed. UFRGS.

van Dijk, K., van der Bom, J. G., Lenting, P. J., de Groot, P. G., Mauser-Bunschoten, E. P., Roosendaal, G., Grobbee, D. E., & van den Berg, H. M. (2005). Factor VIII half-life and clinical phenotype of severe hemophilia A. Haematologica, 90(4), 494–498.

Vlot, A. J., Mauser-Bunschoten, E. P., Zarkova, A. G., Haan, E., Kruitwagen, C. L., Sixma, J. J., & van den Berg, H. M. (2000). The half-life of infused factor VIII is shorter in hemophiliac patients with blood group O than in those with blood group A. Thrombosis and haemostasis, 83(1), 65–69.

Downloads

Publicado

23/11/2024

Como Citar

LIMA, M. do B. P. L. V.; SIQUEIRA, L. H. de .; MONTALVÃO, S. A. de L.; LORENZETTI, T. H. T.; OLIVEIRA FILHO, A. B. de .; FERREIRA, S. H.; SILVA, L. S. da; OZELO, M. C.; LEMOS, J. A. R. de. Maior reposição de Fator VIII (FVIII) da coagulação em pacientes com Hemofilia A moderada dos grupos sanguíneos não-O. Research, Society and Development, [S. l.], v. 13, n. 11, p. e138131147484, 2024. DOI: 10.33448/rsd-v13i11.47484. Disponível em: https://rsdjournal.org/index.php/rsd/article/view/47484. Acesso em: 26 nov. 2024.

Edição

Seção

Ciências da Saúde