In:
Haemophilia, Wiley, Vol. 20, No. 6 ( 2014-11), p. 771-776
Abstract:
The objective of this study was to evaluate the inhibitor development ( ID ) in previously untreated patients ( PUP s) with severe haemophilia A ( FVIII ≤ 0.01 IU mL −1 ). All Canadian Haemophilia Treatment Centres completed a questionnaire on patients born between September 2005 and August 2010 and followed for up to 7 years. Eligible patients had at least 20 exposure days ( ED ) or had developed an inhibitor. The odds ratio ( OR ) and 95% confidence intervals (95% CI ) for risk factors to develop an inhibitor were estimated using unconditional logistic regression. A total of 99 haemophilia A PUP s were studied. Thirty‐four (34%) developed an inhibitor (24/34 of high titre). Inhibitors developed in 25/63 (40%) patients with a high‐risk mutation. ID was most frequent in Aboriginals (86%). Dose intensity ( IU kg −1 day −1 X number of ED ) at first exposure to factor VIII ( FVIII ) was associated with a crude OR increase of 1.10 (95% CI : 0.99–1.23) with each increase of 100 dose‐intensity units. Haemarthrosis and intracranial bleeding as the indication for first exposure to FVIII concentrate were associated with a crude OR for ID of 7.63 (95% CI : 2.14–27.17) and 5.08 (95% CI : 1.11–23.31) respectively. ID according to FVIII concentrate used was: Advate ® 18/50 (36%), Kogenate FS ® or Helixate FS ® 15/36 (42%), Wilate ® 0/11 and Xyntha ® 1/2. In multivariate analysis, Aboriginal ethnicity ( OR = 11.69; 95% CI : 1.11–122.86) and haemarthrosis ( OR = 4.49; 95% CI : 1.08–18.61) were statistically significant. The cumulative incidence of ID in severe haemophilia A PUP s was 34% and varied according to ethnicity, type of bleeding at first ED , type of FVIII product and dose intensity at first exposure.
Type of Medium:
Online Resource
ISSN:
1351-8216
,
1365-2516
DOI:
10.1111/hae.2014.20.issue-6
Language:
English
Publisher:
Wiley
Publication Date:
2014
detail.hit.zdb_id:
2006344-1
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