Development of an inhibitor in a man with mild haemophilia A

Haemophilia ◽  
2017 ◽  
Vol 23 (5) ◽  
pp. e473-e474 ◽  
Author(s):  
M. S. Evans ◽  
M. E. Eyster
Keyword(s):  
Haemophilia A ◽  
Mild Haemophilia

Management of Haemophilia in Sweden

1976 ◽  
Vol 35 (03) ◽  
pp. 510-521 ◽  
Author(s):  
Inga Marie Nilsson
Keyword(s):  
Factor Viii ◽  
Total Population ◽  
Age Groups ◽  
Factor Ix ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Haemophilia B ◽  
Human Fraction ◽  
Mild Haemophilia

SummaryThe incidence of living haemophiliacs in Sweden (total population 8.1 millions) is about 1:15,000 males and about 1:30,000 of the entire population. The number of haemophiliacs born in Sweden in 5-year periods between 1931-1975 (June) has remained almost unchanged. The total number of haemophilia families in Sweden is 284 (77% haemophilia A, 23% haemophilia B) with altogether 557 (436 with A and 121 with B) living haemophiliacs. Of the haemophilia A patients 40 % have severe, 18 % moderate, and 42 % mild, haemophilia. The distribution of the haemophilia B patients is about the same. Inhibitors have been demonstrated in 8% of the patients with severe haemophilia A and in 10% of those with severe haemophilia B.There are 2 main Haemophilia Centres (Stockholm, Malmo) to which haemophiliacs from the whole of Sweden are admitted for diagnosis, follow-up and treatment for severe bleedings, joint defects and surgery. Minor bleedings are treated at local hospitals in cooperation with the Haemophilia Centres. The concentrates available for treatment in haemophilia A are human fraction 1-0 (AHF-Kabi), cryoprecipitate, Antihaemophilic Factor (Hyland 4) and Kryobulin (Immuno, Wien). AHF-Kabi is the most commonly used preparation. The concentrates available for treatment in haemophilia B are Preconativ (Kabi) and Prothromplex (Immuno). Sufficient amounts of concentrates are available. In Sweden 3.2 million units of factor VIII and 1.0 million units of factor IX are given per year. Treatment is free of charge.Only 5 patients receive domiciliary treatment, but since 1958 we in Sweden have practised prophylactic treatment of boys (4–18 years old) with severe haemophilia A. At about 5-10 days interval they receive AHF in amounts sufficient to raise the AHF level to 40–50%. This regimen has reduced severe haemophilia to moderate. The joint score is identical with that found in moderate haemophilia in the same age groups. For treatment of patients with haemophilia A and haemophilia B complicated by inhibitors we have used a large dose of antigen (factor VIII or factor IX) combined with cyclophosphamide. In most cases this treatment produced satisfactory haemostasis for 5 to 30 days and prevented the secondary antibody rise.

Mild haemophilia A in Iceland: clinical genetic studies of three families with the same mutation

1994 ◽  
Vol 235 (5) ◽  
pp. 443-450 ◽  
Author(s):  
Ó. JENSSON ◽  
S. STENBJERG BERNVIL ◽  
S. JÖNSÖDTTIR ◽  
J. INGERSLEV
Keyword(s):  
Haemophilia A ◽  
Clinical Genetic ◽  
Genetic Studies ◽  
Mild Haemophilia

Diagnosis and management challenges in patients with mild haemophilia A and discrepant FVIII measurements

Haemophilia ◽  
2014 ◽  
Vol 20 (4) ◽  
pp. 550-558 ◽  
Author(s):  
M. Trossaert ◽  
A. Lienhart ◽  
C. Nougier ◽  
M. Fretigny ◽  
M. Sigaud ◽  
...  
Keyword(s):  
Haemophilia A ◽  
Diagnosis And Management ◽  
Mild Haemophilia

Acute myocardial infarction during substitution with recombinant factor VIII concentrate in a patient with mild haemophilia A

2004 ◽  
Vol 92 (08) ◽  
pp. 425-426 ◽  
Author(s):  
Jean-Louis Kerkhoffs ◽  
Douwe Atsma ◽  
Pranobe Oemrawsingh ◽  
Jeroen Eikenboom ◽  
Felix Van der Meer
Keyword(s):  
Myocardial Infarction ◽  
Acute Myocardial Infarction ◽  
Factor Viii ◽  
Recombinant Factor Viii ◽  
Haemophilia A ◽  
Mild Haemophilia

Current dosing practices for perioperative factor VIII concentrate treatment in mild haemophilia A patients result in FVIII levels above target

Haemophilia ◽  
2019 ◽  
Vol 25 (6) ◽  
pp. 960-968 ◽  
Author(s):  
Lisette M. Schütte ◽  
Nils Rooij ◽  
Hendrika C. A. M. Hazendonk ◽  
Ron A. A. Mathôt ◽  
Reinier M. Hest ◽  
...  
Keyword(s):  
Factor Viii ◽  
Haemophilia A ◽  
Concentrate Treatment ◽  
Mild Haemophilia

Clinical assessment of efficacy and safety of DDAVP

2010 ◽  
Vol 30 (S 01) ◽  
pp. S172-S175 ◽  
Author(s):  
W. Miesbach ◽  
S. Krekeler ◽  
O. Dück ◽  
B. Llugaliu ◽  
G. Asmelash ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Adverse Reactions ◽  
Fluid Intake ◽  
Von Willebrand Disease ◽  
Haemophilia A ◽  
Efficacy And Safety ◽  
Drug Reactions ◽  
Clinical Routine ◽  
Von Willebrand ◽  
Mild Haemophilia

SummaryThe efficacy of DDAVP (1-deamino-8-D-argi-nine-vasopressin, desmopressin) in mild haemophilia A and von Willebrand disease (VWD) has been established and the use of this well tolerated drug has become clinical routine. In case of increased fluid intake and based on very rarely occurring hyponatraemia, the indication of administration of DDAVP intravenously (i. v.) has to be performed diligently in elderly patients and in children below the age of five years. Aim, patients: Due to clinical practice we were interested in finding prospective parameter potentially correlating with adverse reactions of DDAVP and initiated this study. From 2007 to 2008, we included 49 patients suspicious to suffer from mild haemophilia A (n = 1) or VWD (n = 48) and investigated efficacy and safety of DDAVP after intravenous administration (mean: 0.29 ± 0.032 μg/kg body weight). They underwent clinical and laboratory investigation and were questioned with regard to potential adverse reactions immediately and three days after administration of DDAVP.: Results, conclusion: Most adverse reactions were mild and no serious adverse drug reactions were either observed or reported by the subjects. We identified significant changes of heart rate, blood pressure and leucocytes after conduct of the DDAVP test. The value of these findings has to be investigated in later prospective randomized studies. Further research on identification of prospective parameter is currently ongoing.

Tyr346→Cys mutation results in factor VIII:C assay discrepancy and a normal bleeding phenotype – is this mild haemophilia A?

Haemophilia ◽  
2007 ◽  
Vol 0 (0) ◽  
pp. 071124115610001-??? ◽  
Author(s):  
H. Lyall ◽  
M. Hill ◽  
J. Westby ◽  
C. Grimley ◽  
G. Dolan
Keyword(s):  
Haemophilia A ◽  
Mild Haemophilia

Age-dependent increase of FVIII:C in mild haemophilia A

Haemophilia ◽  
2009 ◽  
Vol 15 (5) ◽  
pp. 1022-1026 ◽  
Author(s):  
W. MIESBACH ◽  
S. ALESCI ◽  
S. KREKELER ◽  
E. SEIFRIED
Keyword(s):  
Haemophilia A ◽  
Age Dependent ◽  
Mild Haemophilia
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