The thrombin generation assay distinguishes inhibitor from non-inhibitor patients with severe haemophilia A.
Haemophilia
; 22(4): e286-91, 2016 Jul.
Article
em En
| MEDLINE
| ID: mdl-27166132
ABSTRACT
INTRODUCTION:
Patients with haemophilia A (HA) have impaired thrombin generation (TG) capacity and TG assay (TGA) values are linearly related to plasma factor VIII (FVIII) levels.AIM:
This study carried out in patients with unmeasurable FVIII (<1 IU dL(-1) ) was aimed at unravelling any difference in TG capacity in patients with or without inhibitors.METHODS:
Blood samples were collected from patients in a non-bleeding state, after a 5-day wash-out period from last treatment.RESULTS:
TGA was performed in 102 patients with severe HA (15% with high-responding inhibitors; 51% with null F8 mutations, that as expected were more prevalent in inhibitor than in non-inhibitor patients). TG capacity was significantly lower in inhibitor than non-inhibitor patients and in those with null mutations than in those with non-null mutations. When the TG capacity was evaluated only in patients with null mutations with and without inhibitors it was lower in the presence of inhibitors.CONCLUSIONS:
This study shows a greater TG impairment in inhibitor patients irrespective of FVIII levels, inhibitor titre and F8 mutation type, suggesting a role for the TGA in unravelling functional interferences of anti-FVIII inhibitors on coagulation system activation.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Trombina
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Hemofilia A
Limite:
Adult
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Humans
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Male
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Middle aged
Idioma:
En
Ano de publicação:
2016
Tipo de documento:
Article