Overall hemostasis potential and aPTT-clot waveform analysis as powerful laboratory diagnostic tools for identification of hemophilia A patients with unexpected bleeding phenotype.
Int J Lab Hematol
; 43(2): 273-280, 2021 Apr.
Article
em En
| MEDLINE
| ID: mdl-32964648
ABSTRACT
INTRODUCTION:
Traditionally used laboratory methods do not always accurately reflect bleeding severity in hemophilia A (HA) patients. The ability of three global assays for identifying patients with unexpected bleeding phenotype was investigated.METHODS:
Overall hemostasis potential (OHP), aPTT-clot waveform analysis (aPTT-CWA), endogenous thrombin potential (ETP), FVIII activities, and prothrombin fragment 1 + 2 concentrations were measured in 62 HA patients (30 severe and 32 non-severe) and 27 male controls. Bleeding phenotype was determined using our proposed scoring system including age at first joint bleed, number of target joints, and number of joint/muscle bleeds per year. Bleeding score ≤ 4 defined patients with mild bleeding phenotype (N = 27); score ≥ 5 defined severe bleeding phenotype (N = 35).RESULTS:
The receiver operating characteristic analysis performed for distinguishing patients with severe and mild bleeding phenotype yielded following values of area under the curve 0.910 (FVIII); 0.891 (aPTT-CWA parameter DELTA); 0.769 (OHP); and 0.634 (ETP). Unexpected bleeding phenotype was identified in 11/62 HA patients 8/32 (25%) non-severe HA patients had severe, while 3/30 (10%) severe HA patients had mild bleeding phenotype, and global assays enabled the identification of all these patients. OHP and DELTA were revealed as the most reliable parameters for bleeding phenotype determination (10/11 and 9/11 unexpected results, respectively).CONCLUSION:
This study emphasizes OHP and aPTT-CWA as a powerful laboratory diagnostic tool in identifying HA patients with unexpected bleeding presentations, with the best results achieved by combining both assays. Global assays should not completely replace FVIII activity measurement but should be a part of the HA diagnostic algorithm.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Tempo de Tromboplastina Parcial
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Coagulação Sanguínea
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Hemofilia A
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Hemorragia
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Hemostasia
Tipo de estudo:
Diagnostic_studies
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Observational_studies
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Prognostic_studies
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Risk_factors_studies
Limite:
Adolescent
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Adult
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Aged
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Int J Lab Hematol
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Croácia