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Blood, 1 June 2007, Vol. 109, No. 11, pp. 4648-4654.
Prepublished online as a Blood First Edition Paper on February 8, 2007; DOI 10.1182/blood-2006-11-056291.
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Submitted November 7, 2006
Accepted February 2, 2007
Treatment-related risk factors of inhibitor development
in previously untreated patients with hemophilia A: the
CANAL cohort study
Samantha C. Gouw, Johanna G. van der Bom*, and H. Marijke van den Berg
Van Creveldkliniek, University Medical Center Utrecht, Utrecht, Netherlands
Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, Netherlands
Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, Netherlands
* Corresponding author; email: j.g.vanderbom{at}lumc.nl.
The CANAL Study (Concerted Action on Neutralizing Antibodies in severe hemophilia A) was designed to describe the relationship between treatment characteristics and inhibitor development in previously untreated patients with severe hemophilia A. This multi-center retrospective cohort study investigated 366 consecutive patients born 1990-2000. The outcome was clinically relevant inhibitor development, defined as the occurrence of at least two positive inhibitor titers combined with a decreased recovery. Eighty-seven (24%)patients developed inhibitors (69 high-titer (19%)). The incidence of inhibitors appeared to be associated with age at first treatment, decreasing from 41% for those treated within the first month of age to 18% in those treated after 18 months; after adjustment for treatment intensity, this association largely disappeared. Surgical procedures and peak treatment moments at start of treatment increased inhibitor risk (relative risk (RR) 3.7, 95% confidence interval (CI) 2.0-7.1 and 3.3, CI 2.1-5.3, respectively). Regular prophylaxis was associated with a 60% lower risk than on demand treatment (RR 0.4, CI 0.2-0.8). Our findings suggest that the previously reported associated between an early age at first exposure and the risk of inhibitor development is largely explained by early, intensive treatment. The latter appears to be an independent risk factor for inhibitor development. In addition, early, regular prophylaxis may protect patients with hemophilia against the development of inhibitors.
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