The Incidence of Factor VIII and Factor IX Inhibitors in the Hemophilia Population of the UK and Their Effect on Subsequent Mortality, 1977–99*

Darby, Sarah C.; Keeling, David; Spooner, R. J. D.; Kan, Sau Wan; Giangrande, Paul; Collins, Peter William; Hill, F. G. H.; Hay, C. R. M.

doi:10.1053/j.seminhematol.2005.12.004

Cited by 137 publications

(185 citation statements)

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“…Mortality associated with the development of inhibitors has change through years, in severe haemophiliacs without HIV, inhibitor development doubled mortality during 1977-1992 in the UK, but during 1993-1999 mortality was identical with and without inhibitors. In severe haemophiliacs without HIV but with inhibitors, mortality from causes involving bleeding decreased during 1977-1999 (P = 0.001) as did mortality involving intracranial haemorrhage (P = 0.007), these results do not appear to be related with the type of treatment for haemophilia or the treatment for the inhibitors [38].…”

Section: Summary Of Findings On Treatmentmentioning

confidence: 89%

The History and Evolution of the Clinical Effectiveness of Haemophilia Type A Treatment: A Systematic Review

Castro

Briceño

Casas

et al. 2012

Indian J Hematol Blood Transfus

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First evidence of cases of haemophilia dates from ancient Egypt, but it was when Queen Victoria from England in the 19th century transmitted this illness to her descendants, when it became known as the ''royal disease''. Last decades of the 20th century account for major discoveries that improved the life expectancy and quality of life of these patients. The history and evolution of haemophilia healthcare counts ups and downs. The introduction of prophylactic schemes during the 1970s have proved to be more effective that the classic on-demand replacement of clotting factors, nevertheless many patients managed with frequent plasma transfusions or derived products became infected with the Human Immunodeficiency Virus (HIV) and Hepatitis C virus during the 1980s and 1990s. Recombinant factor VIII inception has decreased the risk of blood borne infections and restored back longer life expectancies. Main concerns for haemophilia healthcare are shifting from the pure clinical aspects to the economic considerations of long-term replacement therapy. Nowadays researchers' attention has been placed on the future costs and cost-effectiveness of costly long-term treatment. Equity considerations are relevant as well, and alternative options for less affluent countries are under the scope of further research. The aim of this review was to assess the evidence of different treatment options for haemophilia type A over the past four decades, focusing on the most important technological advances that have influenced the natural course of this ''royal disease''.

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Section: Summary Of Findings On Treatmentmentioning

confidence: 89%

The History and Evolution of the Clinical Effectiveness of Haemophilia Type A Treatment: A Systematic Review

Castro

Briceño

Casas

et al. 2012

Indian J Hematol Blood Transfus

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“…Several reports have suggested an increased incidence of clinically significant intracranial hemorrhage and worse outcomes for patients with hemophilia and inhibitors. [24][25][26][27][28][29] Our study was not powered to detect the benefit of prophylaxis for low-incidence, high-morbidity bleeding events such as intracranial hemorrhage. However, it is reasonable to assume that patients who have a good response to prophylaxis are also likely to have a reduced risk of life-threatening bleeding.…”

Section: Discussionmentioning

confidence: 99%

Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors

et al. 2011

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“…It is also generally accepted that inhibitor screening should occur before invasive procedures and at regular intervals during the initial 50 treatment days as this is the highest risk period for inhibitor development. 1 After a patient has received factor for 150 treatment days, the rate of inhibitor development is substantially reduced. 3 Although rates of detection are low with routine surveillance in those with greater than 150 treatment days, we recommend annual testing to facilitate postmarketing surveillance by the Centers for Disease Control and Prevention Universal Data Collection (UDC) Project and other national surveillance programs.…”

Section: How Are Inhibitors Detected?mentioning

confidence: 99%

How we treat a hemophilia A patient with a factor VIII inhibitor

Kempton

White

2009

Blood

151

144

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The most significant complication of treatment in patients with hemophilia A is the development of alloantibodies that inhibit factor VIII activity. In the presence of inhibitory antibodies, replacement of the missing clotting factor by infusion of factor VIII becomes less effective. Once replacement therapy is ineffective, acute management of bleeding requires agents that bypass factor VIII activity. Long-term management consists of eradicating the inhibitor through immune tolerance. Despite success in the treatment of acute bleeding and inhibitor eradication, there remains an inability to predict or prevent inhibitor formation. Ideally, prediction and ultimately prevention will come with an improved understanding of how patientspecific and treatment-related factors work together to influence anti-factor VIII antibody production. (Blood. 2009;113: 11-17)

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The Incidence of Factor VIII and Factor IX Inhibitors in the Hemophilia Population of the UK and Their Effect on Subsequent Mortality, 1977–99*

Cited by 137 publications

References 1 publication

The History and Evolution of the Clinical Effectiveness of Haemophilia Type A Treatment: A Systematic Review

The History and Evolution of the Clinical Effectiveness of Haemophilia Type A Treatment: A Systematic Review

Anti-Inhibitor Coagulant Complex Prophylaxis in Hemophilia with Inhibitors

How we treat a hemophilia A patient with a factor VIII inhibitor

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