E-ISSN: 1308-5263
Extended Half-Life (EHL) Coagulation Factors: A New Era in the Management of Haemophilia Patients [Turk J Hematol]
Turk J Hematol. Ahead of Print: TJH-33427 | DOI: 10.4274/tjh.galenos.2019.2018.0393  

Extended Half-Life (EHL) Coagulation Factors: A New Era in the Management of Haemophilia Patients

M. Cem Ar1, Can Balkan2, Kaan Kavaklı2
1Department of Internal Medicine, Division of Haematology, Istanbul University, Cerrahpaşa Faculty of Medicine, Istanbul; Members of Executive Board of Haemophilia Scientific Subcommittee, Turkish Society of Haematology
2Department of Paediatrics, Division of Haemato- Oncology, Ege University Faculty of Medicine, Izmir, Turkey; Members of Executive Board of Haemophilia Scientific Subcommittee, Turkish Society of Haematology

Despite effective factor replacement and various treatment schedules, there remain several challenges and unmet needs in the prophylactic treatment of haemophilia limiting its adoption and thereby posing an increased risk of spontaneous bleeding. In this regard, extended halflife (EHL) recombinant factor VIII (rFVIII) and factor IX (rFIX) products promise optimal prophylaxis by decreasing the dose frequency, increasing the compliance and improving the quality of life without compromising on safety and efficacy. EHL products might lead to higher trough levels without increasing infusion frequency, or would facilitate ability to maintain trough levels while reducing infusion frequency. This paper aims to provide a comprehensive review on the rationale for developing EHL coagulation factors and their utility in the management of haemophilia, with special emphasis on optimal technique for half-life extension and criteria for defining EHL coagulation factors, as well as indications, efficacy and safety issues of the currently available EHL-rFVIII and EHL-rFIX products. Potential impact of these factors on quality of life, health economics and immune tolerance treatment will be also be discussed alongside the challenges in pharmacokinetic driven prophylaxis and difficulties in monitoring the EHL-products with laboratory assays.

Keywords: Haemophilia, factor replacement therapy; extended half-life products; laboratory assays; pharmacokinetics; quality of life




Corresponding Author: M. Cem Ar, Türkiye


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Impact Factor (2017) = 0.650