title: Thrombin generation testing in haemophilia comprehensive care centres.
abstract: Haemophilia comprehensive care centres (HCCC) were first created more than 50 years ago. Their first objective was educating the patient and healthcare professionals in the management of bleeding. Today HCCCs are centres of excellence with multidisciplinary specialists, which continue to provide essential services that are continually reassessed in light of new scientific information. In addition, HCCCs make significant research contributions by studying new methods to improve the well-being of patients with haemophilia. Laboratory expertise is one of the central pillars of HCCCs with a direct impact on diagnosis and management of the haemophilia disease. Vast efforts have been made for the standardization of factor VIII (FVIII) and FIX measurements and inhibitor detection. Molecular biology has improved diagnostics and made it possible to develop new, more secure FVIII and FIX concentrates for replacement therapy. However, phenotyping of each haemophilia patient with an accurate prediction of the individual bleeding risk and also the individual response of patients to antihaemophilic treatment still remains a challenge. In the last 5 years, an expanding interest of haematologists for thrombin generation testing (TGT) reflects the need for new laboratory tools able to evaluate the overall coagulating capacity of patients. This study will review unmet laboratory needs in haemophilia and the potential applications of TGT in the management of haemophiliacs. Furthermore, technical and standardization issues of the method will be discussed.
authors.affiliation: Unité d'Hémostase Clinique, Hôpital Edouard Herriot, Lyon, France. ydargaud@univ-lyon1.fr
authors.forename: Y
authors.initials: Y
authors.lastname: Dargaud
authors.forename: C
authors.initials: C
authors.lastname: Negrier
mesh_terms: D001779:Blood Coagulation Factors / Q000627:therapeutic use*; D001780:Blood Coagulation Tests / Q000592:standards*; D003191:Comprehensive Health Care / Q000592:standards*; D006467:Hemophilia A / Q000097:blood* / Q000175:diagnosis / Q000628:therapy; D002836:Hemophilia B / Q000097:blood* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007753:Laboratories / Q000592:standards*; D010641:Phenotype
chemical_list: D001779:Blood Coagulation Factors