The role of the laboratory in diagnosis and management of inhibitory antibodies in haemophilia

“…If the results were not within 10%, then they were considered to demonstrate type 2 kinetics (non‐linear) 13 . All inhibitor titres were calculated using the residual activity closest to 50% 14 …”

Section: Methodsmentioning

confidence: 99%

Cross‐reacting recombinant porcine FVIII inhibitors in patients with acquired haemophilia A

et al. 2020

View full text Add to dashboard Cite

Acquired haemophilia A (AHA) is a rare haemostatic disorder caused by the production of autoantibodies to clotting factor VIII (FVIII). These antibodies primarily bind to the A2, C1 or C2 domains of endogenous FVIII, neutralizing the cofactor function. 1,2 The incidence of AHA is estimated at 1.48 per million population per year, of which 80%-90% of patients are elderly, 3 although there is also a reported association with pregnancy. 4 Patients with AHA often present with spontaneous bleeding and extensive soft tissue haematoma 3 with a high risk of morbidity or mortality. 5 Many patients require treatment of active bleeding which may include the use of FVIII bypassing agents, activated prothrombin complex concentrate (APCC), recombinant activated factor VII (rFVIIa) or, more recently, recombinant

show abstract

“…4,10 Other mild bleeding disorders, such as type 1 von Willebrand disease, are not associated with severe bleeding complications, such as ICH, in newborns. 11 It may be possible, depending on the clinical circumstance and parent preference, to manage pregnancies involving these mild bleeding disorders without all the special considerations described here.…”

Section: Introductionmentioning

confidence: 99%

A Practical Guide to the Management of the Fetus and Newborn With Hemophilia

Moorehead

Chan

Lemyre

et al. 2018

Clin Appl Thromb Hemost

View full text Add to dashboard Cite

Newborns with hemophilia are at risk of intracranial hemorrhage, extracranial hemorrhage, and other bleeding complications. The safe delivery of a healthy newborn with hemophilia is a complex process that can begin even before conception, and continues throughout pregnancy, birth, and the newborn period. This process involves the expectant parents and a wide variety of health-care professionals: genetic counselors, obstetricians, neonatologists, pediatricians, radiologists, adult and pediatric hematologists, and nurses with expertise in hemophilia. Because of this multidisciplinary complexity, the relative rarity of births of newborns with hemophilia, and the lack of high-quality evidence to inform decisions, there is considerable variation in practice in this area. We present a comprehensive multidisciplinary approach, from preconception counseling to discharge planning after birth, and describe available options for management decisions. We highlight a number of areas of important uncertainty and controversy, including the preferred mode of delivery, the appropriate use and timing of neuroimaging tests, and the appropriate use of clotting factor concentrates in the newborn period. While the approach presented here will aid clinicians in planning and providing care, further research is required to optimize the care of newborns with hemophilia.

show abstract

The role of the laboratory in diagnosis and management of inhibitory antibodies in haemophilia

Cited by 6 publications

References 37 publications

Guidelines on the laboratory aspects of assays used in haemostasis and thrombosis

Guidelines on the laboratory aspects of assays used in haemostasis and thrombosis

Cross‐reacting recombinant porcine FVIII inhibitors in patients with acquired haemophilia A

A Practical Guide to the Management of the Fetus and Newborn With Hemophilia

Contact Info

Product

Resources

About