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.
The thrombin generation assay distinguishes inhibitor from non-inhibitor patients with severe haemophilia A / M. Mancuso, V. Chantarangkul, M. Clerici, M. Fasulo, L. Padovan, E. Scalambrino, F. Peyvandi, A. Tripodi, E. Santagostino. - In: HAEMOPHILIA. - ISSN 1351-8216. - 22:4(2016 Jul), pp. e286-e291. [10.1111/hae.12927]
The thrombin generation assay distinguishes inhibitor from non-inhibitor patients with severe haemophilia A
M. Fasulo;F. Peyvandi;A. TripodiPenultimo
;
2016
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.File | Dimensione | Formato | |
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