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Blood Coagul Fibrinolysis ; 35(5): 232-237, 2024 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-38700721

RESUMO

BACKGROUND: Predicting the bleeding risk in hemophilia A and B carriers (HAC, HBC) is challenging. OBJECTIVE: The objectives of this study were to describe the bleeding phenotype in HAC and HBC using the standardized Tosetto bleeding score (BS); to determine whether the BS correlates better with factor levels measured with a chromogenic assay than with factor levels measured with chronometric and thrombin generation assays; and to compare the results in HAC and HBC. METHODS: This ambispective, noninterventional study included obligate and sporadic HAC and HBC followed at a hemophilia treatment center between 1995 and 2019. RESULTS AND CONCLUSION: The median BS (3, range 0-21 vs. 3.5, range 0-15, P  = ns, respectively) and the abnormal BS rate (35.6% vs. 38.2%, P  = ns) were not significantly different in 104 HAC and 34 HBC (mean age: 38 years, 6-80 years). However, some differences were identified. The risk of factor deficiency was higher in HBC than HAC. Specifically, Factor VIII activity (FVIII):C/Factor IX activity (FIX):C level was low (<40 IU/dl) in 18.3% (chronometric assay) and 17.5% (chromogenic assay) of HAC and in 47% and 72.2% of HBC ( P  < 0.001). Moreover, the FIX:C level thresholds of 39.5 IU/dl (chronometric assay) and of 33.5 IU/dl (chromogenic assay) were associated with very good sensitivity (92% and 100%, respectively) and specificity (80% for both) for bleeding risk prediction in HBC. Conversely, no FVIII:C level threshold could be identified for HAC, probably due to FVIII:C level variations throughout life.


Assuntos
Hemofilia A , Hemofilia B , Hemorragia , Humanos , Hemofilia A/sangue , Hemofilia A/complicações , Hemofilia B/sangue , Hemofilia B/complicações , Adulto , Adolescente , Criança , Pessoa de Meia-Idade , Hemorragia/etiologia , Hemorragia/sangue , Hemorragia/diagnóstico , Adulto Jovem , Idoso , Masculino , Idoso de 80 Anos ou mais , Feminino , Fator IX/análise , Fator IX/metabolismo , Testes de Coagulação Sanguínea/métodos , Fator VIII/análise
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