Effects of emicizumab on APTT, one-stage and chromogenic assays of factor VIII in artificially spiked plasma and in samples from haemophilia A patients with inhibitors.


Journal

Haemophilia : the official journal of the World Federation of Hemophilia
ISSN: 1365-2516
Titre abrégé: Haemophilia
Pays: England
ID NLM: 9442916

Informations de publication

Date de publication:
May 2020
Historique:
received: 04 11 2019
revised: 26 02 2020
accepted: 17 03 2020
pubmed: 7 4 2020
medline: 15 12 2020
entrez: 7 4 2020
Statut: ppublish

Résumé

Emicizumab (Hemlibra, Roche-Chugai) is a recombinant humanized bispecific IgG4 antibody which mimics some of the actions of activated factor VIII (FVIIIa) by binding to factor X (FX) and activated factor IX (FIXa) to activate FX. To evaluate the effect of emicizumab on the APTT, standard one-stage APTT-based FVIII activity assay (sOSA) using plasma calibrators, modified OSA (mOSA) using r APTT in spiked plasma was performed with 13 APTT reagents and in SHA patients with 5 reagents. OSA in spiked plasma was performed with 9 APTT reagents, 7 APTT reagents were used for OSA in SHA patients and six chromogenic substrate assays (CSA) were performed. In SHA, APTTs normalized after the first dose of emicizumab. At weeks 32/36 of treatment, the mean sOSA FVIII:C ranged from 2.47 IU/mL (Synthasil) to greater than 7.00 IU/mL with all other reagents. mOSA ranged from 59.8 µg/mL (Synthasil) to 74.5 µg/mL (APTT SP). Bovine CSA did not recover any FVIII:C activity. Hyphen Biomed human CSA, demonstrated FVIII activity when calibrated against a plasma calibrator. The APTT was significantly shortened in the presence of emicizumab. sOSA FVIII:C levels were erroneously high, and it is not recommended that these be performed. Quantification of emicizumab concentration was possible by mOSA. Human CSA was sensitive to emicizumab and surrogate FVIII:C activity could be determined. Bovine CSA were insensitive to emicizumab and could not be used to quantify emicizumab concentration.

Identifiants

pubmed: 32249990
doi: 10.1111/hae.13990
doi:

Substances chimiques

Antibodies, Bispecific 0
Antibodies, Monoclonal, Humanized 0
Hemostatics 0
emicizumab 7NL2E3F6K3
Factor VIII 9001-27-8

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

536-542

Subventions

Organisme : Roche

Informations de copyright

© 2020 John Wiley & Sons Ltd.

Références

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Oldenburg J, Mahlangu J, Kim B, et al. Emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med. 2017;377:809-818.
Mahlangu J, Oldenburg J, Paz-Priel I, et al. Emicizumab prophylaxis in patients who have haemophilia A without inhibitors. N Engl J Med. 2018;379:811-822.
Collins PW, Liesner R, Makris M, et al. Treatment of bleeding episodes in haemophilia A complicated by a factor VIII inhibitor in patients receiving emicizumab. Haemophilia. 2018;24:344-347.
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Adamkewicz J, Soeda T, Kotani N, Calatzis A, Levy G. Effect of emicizumab (ACE910) - a humanized bispecific antibody mimicking FVIII cofactor function - on coagulation assays commonly in use for monitoring of hemophilia A patients. Haemophilia. 2017;23:4.
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Turkantoz H, Varnholt D, Tiede A. Monitoring of emicizumab (ACE910): comparison between clotting and chromogenic assay. Hamostaseologie. 2019;39:S1-S10 (abstract).

Auteurs

Annette Bowyer (A)

Department of Coagulation, Sheffield Haemophilia and Thrombosis Centre, Royal Hallamshire Hospital, Sheffield, UK.

Steve Kitchen (S)

Department of Coagulation, Sheffield Haemophilia and Thrombosis Centre, Royal Hallamshire Hospital, Sheffield, UK.

Rhona Maclean (R)

Department of Coagulation, Sheffield Haemophilia and Thrombosis Centre, Royal Hallamshire Hospital, Sheffield, UK.

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